Patients Underwent Bone Marrow Biopsy Research Articles

The Value of Baseline PET/CT Imaging of Bone Marrow 18F-FDG Uptake Pattern in Predicting Prognosis of DLBCL

To investigate the prognostic value of bone marrow uptake pattern in 18F-deoxyglucose (18F-FDG) PET/CT imaging before diffuse large B-cell lymphoma (DLBCL) treatment. The clinical data of 156 patients with DLBCL were retrospectively analyzed. All patients underwent bone marrow biopsy, bone marrow smear, flow cytometry and 18F-FDG PET/CT scan before treatment. Taking normal liver 18F-FDG uptake as the standard, the bone marrow uptake patterns of patients were divided into three types: focal increased bone marrow uptake (fPET+), diffusely increased bone marrow uptake (dPET+), and normal bone marrow uptake (nPET). Survival analysis was performed using the Kaplan-Meier method, log-rank test was used for comparison of differences between groups, and multivariate Cox regression analysis was used to identify risk factors associated with prognosis. Among the 156 patients, 17 cases were fPET+, 28 cases were dPET+, and 111 cases were nPET. Clinical diagnosis of bone marrow infiltration (BMI) was positive in 21 cases and negative in 135 cases. There were 62 cases of recurrence and progression, and 18 cases of death. Univariate analysis showed that Ann Arbor stage III/IV, B symptoms, NCCN-IPI score, lactate dehydrogenase (LDH), BMI+ and fPET+ were associated with progression-free survival (PFS) (all P < 0.05), while Ann Arbor stage III/IV, NCCN-IPI score, LDH, BMI+ and fPET+ were associated with overall survival (OS) (all P < 0.05). Multivariate analysis showed that Ann Arbor stage III/IV, LDH and fPET+ were independent predictors of PFS (all P < 0.05). There were no independent predictors of OS in multivariate analysis. The bone marrow uptake pattern of 18F-FDG imaging in DLBCL patients before treatment has a predictive value for DLBCL, while fPET+ is an independent risk factor for PFS.

The effectiveness of non-pharmacological interventions on reducing pain in patients undergoing bone marrow aspiration and biopsy: A systematic review and meta-analysis of randomized controlled trials.

Patients often consider bone marrow aspiration and biopsy to be one of the most painful medical procedures. The effectiveness of non-pharmacological interventions to reduce pain during bone marrow aspiration and biopsy remains unclear. To synthesize existing evidence regarding the effectiveness of non-pharmacological interventions in mitigating procedural pain among patients undergoing bone marrow aspiration and biopsy. A systematic review and meta-analysis of randomized controlled trials. Six electronic databases, including PubMed, EMBASE, CINAHL, PsycINFO, Cochrane Library and Web of Science were searched from inception to July 15, 2023. The risk of bias was assessed using the Cochrane Risk of Bias Tool Version 2.0. Meta-analysis was conducted using STATA 16. The certainty of the evidence was assessed by the GRADE approach. This meta-analysis included 18 studies derived from 17 articles involving a total of 1017 participants. The pooled results revealed statistically significant pain reduction effects using distraction (SMD: -.845, 95% CI: -1.344 to -.346, p < .001), powered bone marrow biopsy system (SMD: -.266, 95% CI: -.529 to -.003, p = .048), and acupoint stimulation (SMD: -1.016, 95% CI: -1.995 to -.037, p = .042) among patients undergoing bone marrow aspiration and biopsy. However, the pooled results on hypnosis (SMD: -1.228, 95% CI: -4.091 to 1.515, p = .368) showed no significant impact on pain reduction. Additionally, the pooled results for distraction did not demonstrate a significant effect on operative anxiety (MD: -2.942, 95% CI: -7.650 to 1.767, p = .221). Distraction, powered bone marrow biopsy system and acupoint stimulation are effective in reducing pain among patients undergoing bone marrow aspiration and biopsy. Not applicable. This meta-analysis highlights the effectiveness of distraction, powered bone marrow biopsy system and acupoint stimulation for reducing pain in patients undergoing bone marrow biopsy. Healthcare professionals should consider integrating these interventions into pain management practices for these patients. (PROSPERO): CRD42023422854.

Acute myeloid leukemia with monocytic differentiation (myeloid sarcoma) masquerading as bone-soft tissue malignancy

Abstract Introduction/Objective Myeloid sarcoma is an extramedullary solid neoplasm composed of myeloid precursors with or without maturation. This tumor usually occurs simultaneously or following the onset of acute leukemia. Rarely, it can be the first manifestation of acute myeloid leukemia. We report a case of this rare tumor presenting histologically as a bone sarcoma with features of extraskeletal myxoid chondrosarcoma and myoepithelial carcinoma Methods/Case Report A 76-year-old female was referred to our institution for evaluation of a right foot mass associated with pain and swelling. MRI showed a 2.4 x 1.5x 1.2 cm soft tissue mass along the lateral margin of proximal and mid diaphysis of first metatarsal. The clinical and radiologic differential diagnosis was primary bone-soft tissue malignancy, or infection. Histologically, the biopsies showed sheets and cords of cells in a myxoid background. Cytologically the cells were plasmacytoid, mononuclear, mitotically active with immature chromatin, prominent nucleoli, and moderate to abundant cytoplasm. This morphologic appearance elicited the differential diagnosis of extraskeletal myxoid chondrosarcoma, myoepithelial carcinoma, and plasmacytoma. An initial panel of immunohistochemical stains showed the lesional cells to be negative for S-100, SOX-10, pancytokeratin, desmin, p63, EMA, CAM5.2, and CD138 but positive for CD43. Additional immunohistochemistry showed diffuse reactivity for CD33, and lysozyme; CD163 was focally positive, and the cells were negative for CD20, CD3, CD30, CD99, MUM1, CD138, and CD34. A diagnosis of extramedullary acute myeloid leukemia with monocytic differentiation (myeloid sarcoma) was rendered. Subsequently, patient underwent bone marrow biopsy which showed involvement by acute myeloid leukemia with monocytic differentiation. Results (if a Case Study enter NA) NA Conclusion Myeloid sarcoma is a rare tumor and presentation as an isolated metatarsal mass is highly unusual and exceedingly rare. This can mislead clinicians, radiologists, and pathologists. Pathologists need to keep hematopoietic tumors in their differential diagnosis even when the anatomic sites as well as the morphology are not typical of hematopoietic malignancies.

Diagnostic and prognostic values of flow cytometry in diffuse large B-cell lymphoma with bone marrow involvement

Objective: To analyze the diagnostic and prognostic values of flow cytometry (FC) in diffuse large B cell lymphoma (DLBCL) with bone marrow involvement (BMI). Methods: The clinical data of 412 patients with newly diagnosed DLBCL, including 243 males and 169 females, aged 64 (28-92) years old, in the Department of Hematology at Peking University Third Hospital from December 2012 to June 2022 were retrospectively analyzed. All patients underwent bone marrow biopsy (BMB) and bone marrow FC. The patients with BMI by FC were further detected by fluorescence in situ hybridization (FISH) for gene analysis. The positive rates and consistency of BMI detected by BMB and FC were evaluated. According to the results of BMB and FC examinations, all patients were divided into four groups: the BMB+FC+group (115 cases), the BMB-FC+group (50 cases), the BMB+FC-group (8 cases, the results did not include in statistical analysis because of small sample size), and the BMB-FC-group (239 cases). The clinical features, treatment response rates, 5-year survival rates, and immunophenotype characteristics by FC in different groups were analyzed. Results: Among the 412 patients with DLBCL, the positivity rates of BMB and FC for BMI detection were 29.9% (123/412) and 40.0% (165/412), respectively. Good consistency between BMB and FC was found (Kappa=0.841, P=0.001). The numbers of extranodal involvement≥2, splenomegaly, huge mass, higher Ki-67 score, higher international prognostic index (IPI) score, thrombocytopenia, and elevated lactate dehydrogenase level were more prevalent in the BMB+FC+group than those in the BMB-FC+group and the BMB-FC-group (all P<0.05). The treatment response rate in BMB+FC+group was 63.5% (73/115), which was lower than those in BMB-FC+group (88.0%, 44/50, P=0.048) and BMB-FC-group (90.0%, 215/239, P=0.032), respectively. The 5-year overall survival rates in three groups were (53.6±9.7) %, (72.5±8.6) %, and (75.2±7.6) %, respectively, with a statistically significant difference (P=0.037). According to the FISH results of bone marrow, 102 cases were diagnosed as not otherwise specified (NOS), 48 cases were diagnosed as double hit lymphoma (DHL), and 15 cases were diagnosed as triple hit lymphoma (THL). Compared with NOS subtypes, the tumor cells in DHL or THL subtypes had higher proportion of increased side scatter (SSC), higher positive rates of CD10 expression, CD38 strong expression and CD56 expression, and lower proportion of surface immunoglobulin light chain restriction (all P<0.05). Conclusions: FC is well consistent with BMB in diagnosing DLBCL with BMI. Combined with FISH detection, FC can contribute to the auxiliary diagnosis and risk stratification for DHL and THL, and provide reference for the prognostic evaluation in DLBCL with BMI.

A Comparative Study of the Effect of Dexmedetomidine-Fentanyl and Midazolam-Ketamine Combination on the Level of Sedation in Children undergoing Bone Marrow Biopsy

Background: This study aimed to compare the effect of dexmedetomidine-fentanyl (DF) and midazolam-ketamine (MK) combination on the level of sedation in children undergoing bone marrow biopsy.&#x0D; Methods: This study was a single-blind randomized clinical trial. The patients were divided into two groups of 35. Five minutes before undergoing bone marrow biopsy, the first group underwent sedation with a combination of 0.1 mg/kg midazolam with 1 mg/kg ketamine, and the second group underwent sedation with a combination of 2 μg/kg dexmedetomidine with 1 μg/kg fentanyl. The mean arterial pressure (MAP), heart rate, SpO2, the level of sedation, and the incidence rates of complications were recorded in both groups and compared to each other.&#x0D; Results: There was no significant difference between the two groups in terms of age (P= 0.687), gender (P= 1.00), and weight (P= 0.839). However, there was a significant difference in the average length of stay in recovery (P= 0.015) and surgeon satisfaction (P= 0.000), with a longer recovery period in the midazolam-ketamine (MK) group. The Repeated measures ANOVA showed significant differences in heart rate (P= 0.008), sedation score (P= 0.038), and the percentage of oxygen saturation (P= 0.00) during surgery.&#x0D; Conclusion: The combination of dexmedetomidine and fentanyl (DF) compared to the combination of midazolam and ketamine (MK) can provide more patient sedation and surgeon satisfaction along with more stable hemodynamics for patients undergoing bone marrow biopsy.

Morphological Spectrum of Bone Marrow Changes in Patients of Chronic Kidney Disease: A Cross-sectional Study

Introduction: Bone marrow investigations are crucial for Chronic Kidney Disease (CKD) patients with anaemia because they monitor iron stores and reveal morphological abnormalities. In the context of CKD, the examination of bone marrow plays a crucial role in assessing iron levels in individuals with anaemia, as well as providing valuable insights into the specific morphological alterations that occur in CKD patients. Aim: To determine the bone marrow changes and bone marrow iron storage in the patients with CKD; and to study the association between haematological parameters and different CKD stages. Materials and Methods: This cross-sectional study design enrolled 50 CKD patients in the Department of Pathology at MLB Medical College, Jhansi, Uttar Pradesh, India from December 2021 to November 2022. Each patient underwent bone marrow biopsy examination. The Medonic-M Series, an automated haematological analyser, performed the Complete Blood Count (CBC) with red cell indices. Perl’s staining and the Gale Grading Systems were used for assessing iron storage. Descriptive analysis employed frequency and proportions for categorical variables, while mean and Standard Deviation (SD) were used for continuous variables. One-way ANOVA was used to compare differences between variables. Data was collected, compiled, tabulated, entered into a Microsoft Excel sheet, and analysed using Statistical Package for the Social Sciences (SPSS) version 23.0. Results: The study included 50 cases, with the majority of patients being in their fifth and sixth decades of life. Of the total cases, 28 (56%) were males and 22 (44%) were females. Most patients were in Stage-V CKD (N=28, 56%), followed by Stage-III (N=16, 32%), and Stage-IV (N=6, 12%). Among the haematological variables, the difference in Haemoglobin (Hb) was statistically significant (p-value=0.001) in all CKD stages. Iron absorption was found to be normal in 58% (n=29) of patients and increased in 32.00% (n=16) of cases. A total of 26 (52%) out of 50 CKD patients had normocellular bone marrow biopsy and bone marrow smears. Conclusion: Bone marrow examination is undervalued but may be beneficial for CKD patients with anaemia, erythropoietin resistance, thrombocytopenia, or pancytopenia. This method should be considered in centres, whenever possible.

Correlation between whole skeleton dual energy CT calcium-subtracted attenuation and bone marrow infiltration in multiple myeloma

ObjectivesObjective evaluation of the extent of skeletal marrow involvement in multiple myeloma remains a clinical gap for CT. We aimed to develop a quantitative segmentation pipeline for dual energy CT and to assess whether quantified whole skeleton calcium-subtracted attenuation values correlate with biopsy-derived bone marrow infiltration in multiple myeloma. MethodsConsecutive prospective patients with suspected/established myeloma underwent dual source CT from the skull vertex to proximal tibia. Whole skeleton segmentation was performed for 120 kVp-equivalent images as follows: following Hounsfield unit (HU) thresholding, a Chan-Vese morphological operation was implemented to generate a whole skeleton segmentation mask. This mask was then applied to corresponding whole skeleton material decomposition calcium-subtracted maps, generating whole skeleton HU values. Associations with biopsy-derived bone marrow plasma cell infiltration percentage were assessed with Spearman’s rank correlation; significance was at 5%. Results21 patients (12 females; median (IQR) 67 (61, 73) years) were included; 16 patients had osteolytic bone lesions; 15 patients underwent bone marrow biopsy. Segmentation and quantification were feasible in all patients. Median (IQR) of the average skeletal calcium-subtracted attenuation was −59.9 HU (-66.3, −51.8HU). There was a positive correlation with bone marrow plasma cell infiltration percentage (Spearman’s rho: + 0.79, p < 0.001). ConclusionWhole skeleton calcium-subtracted attenuation is associated with the degree of bone marrow infiltration by plasma cells, providing an objective measure of marrow involvement with the potential to allow earlier detection of disease.

Increased 68Ga-PSMA-11 Bone Marrow and Splenic Uptake in a Case of Erythroid Myelodysplasia.

We describe the case of an 82-year-old man who underwent 68Ga-prostate-specific membrane antigen-11 (68Ga-PSMA-11) positron emission tomography/computed tomography (PET/CT) for the restaging of prostate carcinoma. 68Ga-PSMA-11 PET/CT unexpectedly showed increased tracer uptake homogenously involving the axial and appendicular bone marrow and the spleen, not characteristic for metastatic prostate disease. Further investigations revealed that 2months before the patient underwent bone marrow biopsy, which showed the occurrence of monolinear myelodysplasia.

Hematologic Presentations of COVID-19 Can be Misinterpreted as Acute Myeloid Leukemia

Introduction: COVID-19 infection prompts inflammatory responses and acute lung injury in human beings. Complete blood count with differential is essential investigative tool in its managing. However, very few studies revealed the variations of blood cell morphology in this disease.Case report: We reported a 39-years- old female patient complained of respiratory distress one week prior to hospitalization. The patient suffered from cough, fever, and molecular test was reported positive for COVID-19 infection. Laboratory data revealed severe permanent leukopenia and peripheral blood smear examination showed blastoid cells after remission of respiratory signs. Patient underwent bone marrow biopsy for rule out acute myeloid leukemia. But, on bone marrow sample, only viral cytopathic effects were seen. COVID-19 virus stimulates inflammatory cells to produces various inflammatory cytokines and as a result, viral cytopathic effects on white blood cells is seen. Conclusion: We have described how the characteristic peripheral blood findings of COVID‐19 infection can be misinterpret as acute myeloid leukemia.

Occlusive retinal vasculopathy with macular branch retinal artery occlusion as a leading sign of atypical hemolytic uremic syndrome \u2013 a case report

BackgroundHemolytic Uremic Syndrome (HUS) is a rare disorder characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure, considered within the group of thrombocytic microangiopathies. Ocular complications in HUS are very rare. Here, we report an adult patient who suffered from acute onset of paracentral scotoma, caused by branch retinal artery occlusion (BRAO), as a leading symptom of atypical HUS.Case presentationA 39-year-old healthy male was lately diagnosed with essential hypertension and mild renal impairment. He complained about acute onset of central scotoma in his left eye. Fundus examination revealed marked narrowing of retinal vessels, cotton wool spots and few retinal hemorrhages in both eyes. The patient was diagnosed with bilateral ischemic retinal vasculopathy and acute macular BRAO in his left eye. Workup revealed thrombocytopenia, worsening renal failure. Renal biopsy showed signs of chronic thrombotic microangiopathy. The patient was diagnosed with atypical HUS (aHUS) and started on plasmapheresis, together with eculizumab. As his condition continued to worsen, he was put on renal replacement therapy. Due to a persistent monoclone of IgG1, the patient underwent bone marrow biopsy which revealed Monoclonal Gammopathy of renal significance, triggering a HUS and treatment was initiated accordingly. Two months after initial presentation, the patient developed neovascularization of the optic disc (NVD) in his left eye, and was treated with 3 monthly intravitreal bevacizumab injections with complete regression of the NVD. The patient suffered from myocardial infarction in the later course and was lost for follow-up. He returned 11 months after the last bevacizumab injection because of sudden loss of vision in his left eye caused by a dense vitreous hemorrhage. Biomicroscopy revealed a new NVD in his right eye. The patient underwent panretinal photocoagulation in both eyes with regression of neovascularization. Vision improved and remained 20/20 in both eyes.ConclusionWe present a case report showing retinal ischemia can be linked with aHUS. As clinal diagnosis might be challenging, physicians should be aware of the rare ocular manifestations of this devastating multi-organ disease. In case of retinal ischemia, panretinal photocoagulation should be initiated soon to avoid blinding complications.

Long-Term Sustained Minimal Residual Disease (MRD) Negativity in Patients with Multiple Myeloma Treated with Continuous Lenalidomide Maintenance Therapy: A Clinical and Correlative Phase 2 Study

Long-Term Sustained Minimal Residual Disease (MRD) Negativity in Patients with Multiple Myeloma Treated with Continuous Lenalidomide Maintenance Therapy: A Clinical and Correlative Phase 2 Study

S2444 Diagnosis of a Grave Disease in a Seemingly Asymptomatic Woman: Budd-Chiari With Portal Vein Thrombosis in Setting of Polycythemia Vera

INTRODUCTION: Vascular disorder of the liver are rare in the general population with increased prevalence in patients with cirrhosis and MPD. Up to 50 % of patients with BCS have an associated MPD while 10–15% are classified as idiopathic. Coincidence of BCS and PVT has been reported in 10–15% cases but rarely documented as the presenting finding of PV in the setting of an extensive splanchnic thrombosis. CASE DESCRIPTION/METHODS: A 35-year-old female with a past medical history of one spontaneous miscarriage presented with 1 week of epigastric pain and early satiety. On examination she had marked hepatosplenomegaly with diffuse dull percussion note and shifting dullness. On presentation, the patient was hemodynamically stable. Labs showed ALT 810, AST 499, Tb9.4, Db7.1, ALP 272, IINR 2.6, Albumin 2.9, Hemoglobin/Hct 14.3/42. CT of abdomen/pelvis showed hepatosplenomegaly, markedly enlarged left lobe of the liver, and diffuse ascites suggestive of PVT (Figure 1). Patient was started on heparin however course was complicated by worsening of symptoms, repeat imaging two days afterwards showed extension of the clot to involve hepatic vein and SMV thrombosis extending into the IVC with concerns for early signs of bowel necrosis. Patient underwent an emergent successful extended TIPS, IVC stent, and thrombectomy/thrombolysis of SMV and portal vein with no complications post procedure (Figure 2). Work-up showed positive hypercoagulability workup for JAK2 21%, no further mutations detected. Patient underwent bone marrow biopsy which confirmed the diagnosis of PV. Her symptoms improved over the course of 10 days, was transitioned to oral anti-coagulant. DISCUSSION: Acute PVT can sometimes remain with non-specific symptoms however, the extension of thrombosis is usually what depicts the clinical progression, in addition to use of systemic anti-coagulation which is seldom used in chronic forms of PVT. Several studies showed that JAK2 mutation to be found in almost 35% of all splanchnic vein thrombosis forms, and in 41-59% of patients with BCS. However, it is uncommon to see PV in young patients <40 years of age, in whom only a minority will develop BCS. In our case, the symptoms were imperceptible, yet the diagnosis was severe, which raises the possibility of likely a chronic picture of portal vein thrombosis or BCS. It was extremely challenging given the unclear symptom presentation and critical radiographic findings to provide adequate therapy to control extent of thrombosis without thrombolysis and TIPS.Figure 1.: CT scan of the abdomen pelvis in portal venous phase. (A) Coronal view showing pre TIPS hyper-enhancement of the caudate lobe (red). Thrombus in portal vein (orange). Ascites (blue). (B) After TIPS and IVC placement showing heterogeneous liver nutmeg (black/0 and resolution of ascites (blue).Figure 2.: A) Hepatic venogram showing Classical spider-web appearance of Budd-Chiari (blue arrow). B) percutaneous access of splenic vein doing portal venogram showing occluded/thrombosed portal vein (red arrow). C) stent extending from IVC to PV connected via splenic vein catheterization. D) TIPS (blue arrow) and IVC stent (red arrow).

Severe Aplastic Anemia Presenting as Neutropenic Sepsis

A 40-year-old female with aplastic anemia presented with a gum abscess deteriorating into neutropenic sepsis. Infection is an incredibly rare initial manifestation of aplastic anemia even on a background of significant neutropenia, hence the uniqueness of this case. The patient’s initial complaints were of a subacute history of heavy vaginal bleeding and unexplained bruising, however on examination in the emergency department the patient was also noted to be pyrexial with gingival hyperplasia and a left sided submandibular lymphadenopathy. Initial blood results were phoned through from the lab reporting pancytopenia, confirming clinical suspicion of neutropenic sepsis. Antibiotic therapy was commenced and maxillofacial review for her unrelenting jaw pain revealed a gum abscess ultimately requiring tooth extraction. The patient underwent bone marrow biopsy showing hypocellular marrow with erythroid-dominant, dysplastic hematopoiesis. A thorough panel of investigations to rule out secondary causes led to the diagnosis of aplastic anemia, for which the patient is currently being managed with oral ciclosporin plus eltrombopag-bridging therapy, plus counselling for the potential requirement for stem cell transplant. J Med Cases. 2020;11(11):339-341 doi: https://doi.org/10.14740/jmc3570

The Bruton tyrosine kinase inhibitor ibrutinib improves anti-MAG antibody polyneuropathy

ObjectiveTo assess whether neuropathy with anti-myelin-associated glycoprotein (MAG) antibody may improve after treatment with ibrutinib, an oral inhibitor of Bruton tyrosine kinase, we prospectively treated with ibrutinib a cohort of 3 patients with anti-MAG neuropathy and Waldenström macroglobulinemia (WM).MethodsAll 3 patients underwent bone marrow biopsy showing WM, with MYD88L265P mutated and CXCR4S338X wild type, and were started on ibrutinib 420 mg/die. Patients were assessed at baseline, at 3-6-9 months, and at 12 months in 2 patients with a longer follow-up, using Inflammatory Neuropathy Cause and Treatment (INCAT) Disability Score, INCAT sensory sum score, and Medical Research Council sum score. The modified International Cooperative Ataxia Rating Scale was performed in 2 patients, whereas it was not used in the patient with Parkinson disease as a major comorbidity. Responders were considered the patients improving by at least one point in 2 clinical scales.ResultsAll the patients reported an early and subjective benefit, consistent with the objective improvement, especially of the sensory symptoms as shown by clinical scales. Treatment was well tolerated.ConclusionThese preliminary data point to a possible efficacy of ibrutinib in anti-MAG antibody neuropathy, which is the most common disabling paraproteinemic neuropathy, where active treatment is eagerly needed.Classification of evidenceThis study provides Class IV evidence that for patients with anti-MAG antibody neuropathy, ibrutinib improves neuropathy symptoms.

Effects of Video-Based Information Provision Using a Smart Pad on Patients Undergoing Bone Marrow Biopsy.

The purpose is to determine the effect of video-based information provision using a smart pad on uncertainty, anxiety, physiological parameters, pain, and educational satisfaction among patients hospitalized for a bone marrow biopsy. This study was done with a pre-/posttest nonequivalent control group design. The subjects were 65 patients in the hematology-oncology ward of a university hospital in Cheongju, South Korea, who underwent a bone marrow biopsy between August 2017 and May 2018. Thirty volunteers were allocated to the control group and 35 volunteers to the intervention group. The experimental group showed significantly lower uncertainty and significantly greater satisfaction with education than did the control group. No significant difference was observed in anxiety, systolic and diastolic blood pressure, pulse, and pain. Video-based information provision using a smart pad was effective for lowering uncertainty among patients receiving a bone marrow biopsy, as well as for boosting their sense of educational satisfaction.

Long-Term Sustained Minimal Residual Disease (MRD) Negativity in Multiple Myeloma Patients Treated with Lenalidomide Maintenance Therapy: A Clinical and Correlative Phase 2 Study

Long-Term Sustained Minimal Residual Disease (MRD) Negativity in Multiple Myeloma Patients Treated with Lenalidomide Maintenance Therapy: A Clinical and Correlative Phase 2 Study

Pattern of clinical and hematological profile in patients diagnosed as megaloblastic anemia in a tertiary care centre

Objectives: To determine the pattern of clinical presentation, pattern of cytopenias, and haematological parameters of megaloblastic anemia in our setup. Study Design: Cross Sectional Descriptive study. Setting: Pathology Department, Khyber Teaching Hospital, Peshawar. Duration: January 2016 to December 2016 (one year period). Material and Methods: A total of 337 patients underwent bone marrow biopsy during the study period, out of which 46 patients were diagnosed as having megaloblastic anemia on bone marrow aspirate and B12 levels. The clinical features and hematological parameters of these patients were recorded in a proforma and the results were drawn accordingly. All cell counts were done on automated counter machine. Results: Out of 337 patients referred to pathology department for bone marrow aspiration, about 45 cases had megaloblastic anemia. These 45 cases were included in the study. Age of the study sample ranged from 1 year to 90 years (mean 32.2 years ± 12.1 SD). There were 35 (78%) males and 10 (22%) females, with male to female ratio of 3.5:1. Commonest clinical feature was generalized weakness and loose motion, while commonest physical sign was pallor, which were seen in 36 (80%), 36 (80%) and 40 (88%) cases respectively. About 28 (62%) patients had pancytopenia, 9 (20%) patients had bicytopenia (low hemoglobin and platelet count), and 8 (18%) cases had isolated anemia. Low Hb was present in all the 45 (100%) patients. Both the mean hemoglobin level and platelet count were reduced (7.4g/dL and 128x109/L. respectively) while mean white cell count in study sample was normal (mean 5.0x109/L). Conclusion: Megaloblastic anemia may presents as anemia, bicytopenia or pancytopenia. The cytopenias are important feature of megaloblastic anemia. Generalized weakness, and lethargy are the common clinical presentation, while pallor is common physical finding. Patients presenting with a constellation of generalized weakness, pallor and pancytopenia should be suspected of megaloblastic anemia and should be investigated accordingly.

Allogeneic hematopoietic stem cell transplantation contributes to eradication of minimal residual disease in adult Philadelphia chromosome-positive acute lymphoblastic leukemia patients treated with dasatinib.

7024 Background: Philadelphia Chromosome-Positive (Ph+) disease is associated with a poor prognosis in acute lymphoblastic leukemia (ALL). Recent studies have shown that the eradication of minimal residual disease (MRD) in this population leads to improved survival outcomes. While hematopoietic stem cell transplantation (HSCT) has demonstrated clinical benefits in Ph+ ALL patients treated with the tyrosine kinase inhibitor (TKI) imatinib, its role is less clear with the use of more potent, newer-generation TKIs such as dasatinib. Methods: This was a retrospective study analyzing the impact of allogeneic HSCT on MRD status in Ph+ ALL patients treated with dasatinib. Patients were divided into 2 groups: those treated with chemotherapy plus dasatinib followed by allogeneic HSCT and those who received chemotherapy plus dasatinib alone. All patients underwent bone marrow biopsy with MRD analysis following induction therapy and subsequent re-evaluation of MRD status at day 100 post-transplant in the HSCT group and after further cycles of chemotherapy plus dasatinib in the non-transplant group. MRD-negative disease was defined as the absence of a BCR-ABL1 transcript by real-time quantitative polymerase chain reaction (qRT-PCR) with a sensitivity of 0.01%. Results: A total of 51 adult Ph+ ALL patients with MRD-positive disease following induction therapy were included. Twenty-seven patients (53%) were male and the median age at time of diagnosis was 42 years (range 23-68). There were 29 patients in the transplant group and 22 patients in the non-transplant group. When analyzing rates of MRD eradication, 18 (62%) patients in the transplant group were found to have MRD-negative disease at day 100 post-transplant compared to 7 (32%) patients in the non-transplant group who only received further cycles of chemotherapy plus dasatinib (risk ratio 0.56, 95% confidence interval 0.32-0.96, p = 0.048). Conclusions: In the era of newer-generation TKIs, allogeneic HSCT continues to have notable benefits in Ph+ ALL such as a significantly higher rate of MRD eradication as demonstrated in this study.

SLAMF7high CD16negative Monocytes Increase in Peripheral Blood of Patients with Myelofibrosis in Correlation with JAK2V617F Mutation

IntroductionMyelofibrosis (MF) occurrence can be attributed to various pathogenic mechanisms. A recent study showed that the neoplastic clone of fibrocytes (spindle shaped, fibroblast-like blood cells derived from monocyte lineage) was essential in primary MF pathogenesis; moreover, serum amyloid P (PRM-151), which suppresses fibrocyte differentiation, markedly improved survival and MF in a murine xenograft model (J Exp Med 2016; 213: 1723). Using a romiplostim-induced murine MF model, direct induction of fibrocyte differentiation using TPO receptor activation, leading to MF progression, was demonstrated (Leukemia 2017; 31: 2709).Using DNA microarray analysis, we previously showed that compared with macrophages, human fibrocytes highly express SLAMF7 (J Immunol 2015; 195: 4341). Elotuzumab (Elo) is an anti-SLAMF7 antibody recently developed for treating multiple myeloma. We showed that Elo inhibits human fibrocyte differentiation in vitro and relieves MF using the mouse model in vivo, identifying Elo as a potential therapeutic agent (manuscript in preparation). Additionally, monocytes with high SLAMF7 expression and negative CD16 expression were significantly elevated in the peripheral blood (PB) of MF patients compared with that of healthy controls (HCs). We hypothesized that this monocyte subset reflects fibrocyte activation and is the target of Elo.For the clinical study of Elo, we evaluated SLAMF7high CD16− monocyte percentage in PB of HCs, myeloproliferative neoplasm (MPN) patients with or without MF, and non-MPN patients with MF in a cross-sectional manner.MethodsSix HCs, 12 non-MPN patients with MF, and 44 MPN patients (18 without and 26 with MF) were enrolled; their blood samples were collected. All MPN patients underwent bone marrow biopsy in 2016-2018 and were diagnosed by the 2016 WHO classification and diagnostic criteria. Non-MPN patients underwent bone marrow biopsy in 2015-2018, and MF was confirmed over MF-1 according to the European Consensus Criteria.SLAMF7high CD16- monocyte percentage in PB monocytes of HCs and patients was calculated using flow cytometry. Further, all patients were tested for JAK2V617F, CALR, or MPL genetic mutations. The allele burden of JAK2V617F was measured. Moreover, fibrocytes were differentiated from PB mononuclear cells of patients with genetic mutation and were verified for genetic mutation in their fibrocytes.ResultsPatients were classified in three groups (MPN without MF, MPN with MF, and non-MPN with MF). The median percentage of SLAMF7high CD16− monocytes in PB of HCs, MPN patients without MF, MPN patients with MF, and non-MPN patients with MF were 1.66%, 2.48%, 27.4%, and 19.8%, respectively. Compared with HCs and MPN patients without MF, SLAMF7high CD16− monocyte percentage of MPN and non-MPN patients with MF significantly increased (p < 0.05 and p < 0.01, respectively) (Figure A).MPN patients with MF harboring JAK2V617F (n = 17) had a significantly higher SLAMF7high CD16− monocyte percentage than those without MF harboring JAK2V617F (n = 8) (median: 43.70% vs. 7.00%, p < 0.001). While a similar trend was observed in patients not harboring JAK2V617F (median: 1.15% vs. 3.26%, p = 0.05), the contrast between patients with and without MF patients was significant for those with JAK2V617F (Figure B). More than 25% of SLAMF7high CD16− monocytes in PB indicates MF in MPN patients harboring JAK2V617F (sensitivity: 82.4%, specificity: 87.5%).In addition, the JAK2V617F allele burden of fibrocytes was strongly correlated with the SLAMF7high CD16− monocyte percentage in PB (p = 0.0003) (Figure C). In all patients, fibrocytes and PB harbored the same genetic mutation.ConclusionThe percentage of SLAMF7high CD16− monocytes in PB elevated in MF patients regardless of MPN, whereas it was not elevated in MPN patients without MF. In MPN patients, those with MF harboring JAK2V617F presented a high SLAMF7high CD16-monocyte percentage in PB, positively correlating with the JAK2V617F allele burden of fibrocytes. In conclusion, SLAMF7high CD16- monocyte levels are closely related with MF and neoplastic clones of fibrocytes harboring JAK2V617F, indicating fibrocyte activation and representing a non-invasive marker of MF progression and a potential target for Elo treatment. [Display omitted] DisclosuresUsuki:Sumitomo Dainippon Pharma: Research Funding, Speakers Bureau; Celgene Corporation: Research Funding, Speakers Bureau; Daiichi Sankyo: Research Funding; Boehringer-Ingelheim Japan: Research Funding; Sanofi K.K.: Research Funding; Shire Japan: Research Funding; SymBio Pharmaceuticals Limited.: Research Funding; Chugai Pharmaceutical: Speakers Bureau; Takeda Pharmaceutical: Speakers Bureau; Ono Pharmaceutical: Speakers Bureau; Novartis: Speakers Bureau; Janssen Pharmaceutical K.K: Research Funding; Pfizer Japan: Research Funding, Speakers Bureau; GlaxoSmithKline K.K.: Research Funding; Kyowa Hakko Kirin Co., Ltd.: Research Funding; Otsuka Pharmaceutical Co., Ltd.: Research Funding; Astellas Pharma Inc.: Research Funding; Nippon Shinyaku: Speakers Bureau; Mochida Pharmaceutical: Speakers Bureau; MSD K.K.: Speakers Bureau.

B-Cell Lymphoblastic Leukemia/Lymphoma Presenting as Focal Hepatic Masses

Precursor B-cell lymphoblastic leukemia/lymphoma is a rare hematologic malignancy. The estimated global incidence is 5:100,000 persons per year (1). It usually presents in children with hepatomegaly, splenomegaly and lymphadenopathy. Primary hepatic lesion as a first presentation in adult is uncommon. A 30 year-old Caucasian woman with no past medical history who presented with high-grade fever, chills, and night sweats for 2 weeks. She complained of right upper abdominal pain, nausea and poor appetite. Blood pressure was 113/80 mmHg. Heart rate was 110 beats/minute. Body temperature was 102.1°F. Physical examination revealed icteric sclera, hepatosplenomegaly, and no lymphadenopathy. Complete blood count showed white blood cell of 4,670/UL, platelet counts of 36,000/L and hemoglobin of 8.9 g/dL. Liver function test showed AST 72 U/L, ALT 103 U/L and alkaline phosphatase 501 U/L. Total bilirubin was elevated at 6.3 mg/dL with high direct bilirubin. Further investigation with magnetic resonance cholangiopancreatography revealed 2 round hepatic masses with T1-weighted hypointensity and T2-weighted hyperintensity, size 1.5 cm and 3.2 cm (Figure 1). An ultrasonography-guided biopsy at the hepatic lesion revealed B-cell lymphoblastic leukemia/lymphoma. Subsequently, patient underwent bone marrow biopsy showing 50% lymphoid blasts. Immumohistochemical stains were positive for CD34, TdT, CD10, and CD79a (Figure 2). Flow cytometry from peripheral blood was positive for CD 19, CD22, and CD 79a. Patient was diagnosed with the precursor B-cell acute lymphoblastic leukemia/lymphoma with hepatic involvement. She was treated with hyper-CVAD chemotherapy. Precursor B cell lymphoblastic leukemia/lymphoma (B-ALL/LBL) is a neoplasm of immature B-cell (2). Hepatic involvement of B-ALL/LBL varies from hepatomegaly, hepatic failure or hepatic lesions (3). Abnormal liver function tests can be cholestasis or hepatitis profile. Characteristics of enhancement in computer tomography or magnetic resonance imaging are not specific. The diagnosis is established by immunohistochemical stains showing immature B cell lineage from a tissue biopsy. Systemic chemotherapy is a main treatment for B-ALL/LBL. B-ALL/LBL presenting as a primary hepatic lesion is rare. Hematologic malignancy should be in the differentials in patients presenting with constitutional symptoms and hepatosplenomegaly with a newly found hepatic lesion(s).2427_A Figure 1. A magnetic resonance imaging of abdomen demonstrates a 1.5 cm lesion at right hepatic lobe and a 3.2 cm lesion at left hepatic lob (arrow).2427_B Figure 2. A pathology of the hepatic lesion from an ultrasonography-guided biopsy shows lymphoblasts infiltrating in hepatic sinusoids