Presentation Of Chronic Myeloid Leukemia Research Articles

Chronic myeloid leukaemia presenting as acute pulmonary thromboembolism

Abstract Myeloproliferative neoplasms are associated with increased viscosity and thrombosis most commonly leading to splanchnic vein thrombosis and Budd Chiari syndrome. Pulmonary thromboembolism is a rare possibility. Chronic Myeloid Leukaemia commonly present with anaemia, splenomegaly. In cases presenting with thrombosis, leucocytosis is generally observed. Eosinophilia is also a rare presentation of CML. Here we present a case of young male presented with unprovoked Acute Pulmonary Embolism and eosinophilia. On evaluation found to be having Philadelphia Chromosome positive and improved on treatment with Tyrosine Kinase Inhibitors. This case highlights the importance of consideration of pulmonary embolism as one of cause of acute onset shortness of breath especially when there are suggestive ECG changes and emphasises the need for evaluation of cause of Acute thromboembolism.

P-O13 Spontaneous Splenic Rupture as first presentation of Chronic Myeloid Leukaemia

Abstract Background Although rare, spontaneous splenic rupture (SSR) is a potentially life threatening condition and most commonly associated with infection and malignancy. Haematological malignancies are an important differential diagnosis and among these chronic myeloid leukaemia (CML) has one of the highest associations with SSR. Methods This case report follows a previously fit and healthy thirty-three year old gentleman who presented to accident and emergency with one day history of sudden onset, severe, left upper quadrant pain. CT of the abdomen and pelvis including an arterial phase, confirmed splenic rupture with moderate volume haemo-peritoneum, but no active bleeding. White cell count (WCC) was 225.8 x10^9/l and blood film confirmed suspicion of haematological malignancy. The patient was haemodynamically stable and therefore transferred to the surgical high dependency unit for observation and conservative management. He was commenced on hydroxycarbamide on the advice of haematology. Results Genetic screening detected BCR-ABL1 and Philadelphia chromosome; this along with bone marrow aspirate confirmed diagnosis of CML. His WCC decreased with hydroxycarbamide therapy. He made good clinical progress and was discharged home with haematology follow up. Hydroxycarbamide has since been stopped and he has been initiated on Imatinib therapy. His WCC has returned to normal and he has remained well with no further complications. Conclusions SSR is an extremely rare first presentation of CML. Given its associated morbidity and mortality, it should remain an important differential diagnosis in patients presenting acutely to the General Surgeon with abdominal pain and shock, especially in those with a known underlying haematological malignancy.

Isolated Ocular Manifestations in Chronic Myeloid Leukaemia

Background: Chronic myeloid leukaemia (CML) presenting with only ocular manifestations either at the initial stage of diagnosis or at relapse is uncommon. We report two cases of CML presenting with isolated visual symptoms.Case series: The first case is a 21-year-old healthy gentleman who presented with left eye painless loss of vision for a one-week duration. Visual acuity was 6/60 in the left eye and 6/6 in the right eye. There were scattered retinal haemorrhages in both eyes and a sub-macular bleed over the left eye. The full blood count revealed a high white cell count of 134.6 × 109/L. Peripheral blood smear showed hyper-leucocytosis with absolute eosinophilia and basophilia and the presence of blasts suggestive of CML thus chemotherapy was commenced. The second case is a 28-year-old in haematological, molecular, and cytogenic remission from CML for the past two years, presented with left eye painless vision loss for five days duration. Vision in the left eye was counting fingers. There was a large subretinal mass involving the left optic disc. Magnetic resonance imaging of the brain and orbit showed an elliptical orbital mass at the left globe posteriorly with diffuse thickening of the optic nerve. The patient was diagnosed as CML relapsed to the left optic nerve. He underwent intrathecal chemotherapy and orbital irradiation.Conclusion: Both these cases are unique since the manifestation of CML was with only ocular features at the time of presentation as per in the first case during the initial diagnosis and in the second case during relapse. This highlights that it is evident that the knowledge of ocular involvement in leukaemia is crucial since the eye is the only organ where leukemic infiltration to nerves and blood vessels can be observed directly. Recognizing fundus changes in leukaemia allows earlier diagnosis and prompt treatment. These case reports highlight the importance of recognizing early fundus changes, which should allow earlier diagnosis and treatment.

A Predictive Score for Outcomes of Tyrosine Kinase-Inhibitor Therapy in Persons with Chronic Myeloid Leukaemia Presenting in Accelerated Phase

Background Chronic myeloid leukaemia (CML) presenting in accelerated phase (AP) is uncommon and there are few data on predictive co-variates for outcomes of tyrosine kinase-inhibitor (TKI)-therapy. Moreover, it is unknown which outcomes of TKI-therapy in these persons correlate with European LeukemiaNet (ELN) definitions of warning and failure which were developed in people in chronic phase or whether imatinib and 2 nd generation tyrosine kinase-inhibitors (TKIs) are equally effective.Objective Identify co-variates correlated with outcomes of TKI-therapy in persons with CML presenting in AP diagnosed by MD Anderson or World Health Organization (WHO) criteria and develop a predictive score. Determine which outcomes correlate with ELN criteria of warning and failure. Compare outcomes of initial therapy with imatinib versus 2 nd-generation TKIs.Methods We interrogated data from 312 and 334 consecutive subjects with CML presenting in AP and receiving imatinib or a 2 nd-generation TKI as initial therapy. Diagnosis of AP was based on widely-accepted MD Anderson or WHO criteria. Demographic, clinical and laboratory co-variates significantly correlated with outcomes were analyzed in a Cox multi-variable regression model. Propensity score matching was done to compare outcomes of initial therapy with imatinib versus a 2 nd generation TKIs.Results In the cohort defined by MD Anderson criteria there were 197 males (63%) with a median age of 41 years (Interquartile Range [IQR], 30 - 54 years). 106 subjects (34%) had ≥ 1 co-morbidities. Median haemoglobin concentration was 99 g/L (range, 40-176 g/L), WBC concentration, 138 x 10E+9/L (range, 3-797 x10E+9/L), platelet concentration, 450 x 10E+9/L (range, 11-4094 x10E+9/L) and percentage blood or bone marrow blasts (whichever was higher), 4% (range, 0-27%). Non-mutually exclusive criteria for classifying subjects as AP included: (1) 15-29% blasts (n = 22, 7%); (2) blood basophils ≥ 20% (n = 184, 59%); (3) platelets < 100 × 10E+9/L unrelated to therapy (n = 31, 10%); (4) clonal evolution (n = 45, 15%); (5) ≥ 2 features (n = 30, 10%). Co-variates associated with failure-free survival (FFS) were haemoglobin concentration < 100 g/L (HR = 1.9; 95% Confidence Interval [CI], 1.1, 3.1; p = 0.014) and blasts > 4.5% (HR = 1.8 [1.1 - 2.9]; p = 0.013). Co-variates associated with progression-free survival (PFS) were platelets < 230 × 10E+9/L (HR = 3.3 [1.7, 6.5]; p < 0.001), blasts > 4.5% (HR = 2.4 [1.3, 4.6]; p = 0.007) and ≥ 1 co-morbidities (HR = 2.4 [1.2, 4.7]; p = 0.010). Co-variates associated with survival were haemoglobin concentration < 100 g/L (HR = 3.3 [1.1, 10.2]; p = 0.04), platelets < 230 × 10E+9/L (HR = 11.4 [3.9, 33.3]; p < 0.001) and ≥ 1 co-morbidities (HR = 6.7 [2.3, 19.5]; p < 0.001). Next, we divided subjects into 4 cohorts: (1) low-risk (no adverse co-variate; n = 49); (2) intermediate-1 risk (1 adverse co-variate; n = 116); (3) intermediate-2 risk (2 adverse co-variates; n = 92); and (4) high-risk (≥ 3 adverse co-variates; n = 47) with significant different probabilities of FFS, PFS and survival (all p-values < 0.001). Using the 2020 ELN criteria for w arning at 3 months was significantly-associated with worse FFS (HR = 3.1 [1.7, 5.7]; p < 0.001). Failure at 3 months was significantly associated with worse PFS (HR = 9.3 [4.3, 18.8]; p < 0.001) and survival (HR = 6.2 [2.0, 19.2]; p = 0.002). In propensity score matching analyses subjects receiving initial therapy with imatinib had lower probabilities of complete cytogenetic response (CCyR; HR = 1.3 [1.0, 1.8]; p = 0.079), major molecular response (MMR; HR = 1.2 [0.8, 1.7; p = 0.386) and molecular response 4.5 (.5; HR = 1.8 [1.1, 3.1]; p = 0.019). However, other endpoints including FFS, PFS and survival were similar for both interventions. Similar results in the subjects diagnosed as AP using the WHO criteria.Conclusions We identify co-variates associated with several outcomes of TKI-therapy in persons presenting in AP CML and used these to develop a prognostic score. We show the 2020 ELN criteria for warning and failure to TKI-therapy developed in persons in chronic phase also operate in subjects diagnosed in AP. Lastly, using propensity score matching we show that whilst some landmarks are achieved more rapidly in persons initially treated with a 2 nd generation TKI, FFS, PFS and survival are similar to those in persons initially treated with imatinib. Our data should help inform physicians treating person with CML presenting in AP. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Blast phase of chronic myeloid leukemia presenting as early T-cell precursor lymphoblastic leukemia.

Blast phase of chronic myeloid leukemia presenting as early T-cell precursor lymphoblastic leukemia.

Rare presentation and initial management of ischemic priapism in two cases of chronic myeloid leukemia

Priapism is a rare clinical presentation in patients with chronic myeloid leukemia. Treatment involves multidisciplinary team efforts of the urologist and haemato-oncologist. We report two cases of priapism: a 20-year-old male with chronic myeloid leukemia who presented with priapism as a component of hyperviscosity syndrome and a 26-year-old previously healthy, non-sexually active male who had priapism as his first presentation of chronic myeloid leukemia, and the role of specialists in the management of these patients.

A Case of Chronic Myeloid Leukemia Presenting with Osteoid Osteoma: A Diagnostic Dilemma

Chronic myeloid leukemia is a hematological malignancy resulting from a clonal proliferation of hematopoietic stem cells of the myeloid series. It is developed by chromosomal translocation. Chronic Myeloid Leukemia (CML) usually occurs in adults and is rare in children. Osteoid osteoma is a benign bone lesion with no malignant potential. The association of chronic myeloid leukemia with osteoid osteoma is not reported in the literature. An atypical presentation of osteoid osteoma may complicate the diagnosis when associated with a primary malignancy. In this report, we have discussed a case of atypical presentation of osteoid osteoma suspected to be a lytic bone lesion in CML.

Pediatric Chronic Myeloid Leukemia with Megakaryocytic Blast Crisis as Initial Presentation: Case Report and Review of Literature

Abstract: Background: Pediatric Chronic Myeloid Leukemia (CML) is a rare entity accounting for 2-3% of pediatric malignancies. CML rarely presents as Blast Crisis (BC) at the time of diagnosis, and megakaryocytic blast crisis is even rarer. Case Presentation: We herein, report a case of a young female, 10-year-old who presented with anemia, leukocytosis and massive splenomegaly. Clinical features, peripheral film and bone marrow findings were consistent with CML in megakaryocytic blast crisis. Bone marrow cytogenetic analysis revealed karyotype of 46, XX, t(9:22)(q34;q11.2) in 20 metaphases and BCR-ABL P210 by PCR was detected with transcript level of 83%, which further confirmed our diagnosis. Conclusion: De novo presentation of chronic myeloid leukemia with megakaryocytic blast crisis is rarely observed in pediatric population with very few cases published till now. We are presenting this case because of its rarity, likelihood of misdiagnosis as AML (M7) and poor prognosis, if not treated precisely. Keywords: Chronic Myeloid Leukemia (CML), Acute Myeloid Leukemia (AML), Blast Crisis (BC), Acute Megakaryocytic Leukemia (AMKL), Chronic Phase (CP), Accelerated Phase (AP), Tyrosine Kinase Inhibitor (TKI).

Headache and Subdural Hematoma as Initial Presentation of Chronic Myeloid Leukemia: A Case Report

Headache and Subdural Hematoma as Initial Presentation of Chronic Myeloid Leukemia: A Case Report

Unilateral disc edema as the first presentation of chronic myeloid leukemia.

Unilateral disc edema as the first presentation of chronic myeloid leukemia.

Chronic myeloid leukaemia presenting with bilateral papilloedema.

Chronic myeloid leukaemia presenting with bilateral papilloedema.

Chronic myeloid leukaemia presenting as acute small bowel gangrene: A case report

Rationale: Chronic myeloid leukaemia is a myeloproliferative disorder due to clonal hyperproliferation of myeloid cells within the bone marrow. It can present both pro- and anti-thrombotic states. CML has different presentations within the gastrointestinal tract.Patient’s concern: A 40-year-old non-diabetic and non-hypertensive male complained of abdominal pain with nausea and emesis for 1 day. Besides, he had a history of abdominal distension and fever for 1 day.Diagnosis: Acute small bowel gangrene due to chronic myeloid leukaemia.Intervention: A limited resection of small intestine with ileostomy and mucus fistula.Outcome: After 3 months following surgery the patient underwent stoma closure. The patient was followed up for more than 3 years postoperatively. During the follow-up, the patient was asymptomatic without any recurrence of the disease.Lesson: Chronic myeloid leukaemia should be considered as one of the causes for small intestine gangrene when there is increased leukocyte count, splenomegaly without evidence of atherosclerotic occlusion or systemic emboli from the heart.

A case of chronic myeloid leukemia presenting as oral ulcers.

The oral signs and symptoms may reflect undetected serious systemic diseases. Depending on the oral manifestation, the dentists and physicians make attention and focusing on specific diagnoses. Here, we represent one such case which was diagnosed after oral signs and symptoms followed by peripheral smear report as chronic myeloid leukemia. Leukemia is among the most prevalent neoplasia, which represents between 30% and 51% of that total. Leukemia is characterized by uncontrolled production of immature white blood cells, causing a series of clinical and oral manifestations, which are important in disease diagnosis. Due to their clinical importance, all such lesions deserve the full attention of dental doctors. Early detection of leukemia is very important because it provides a favorable prognosis.

An interesting case of chronic myeloid leukemia presenting as B-cell lymphoblastic crisis

We present the case of a 46-year-old male patient who came to our emergency department in December 2019 for pain in the left abdominal quadrant. The patient had no fever, night sweats or a history of weight loss. The la­bo­ra­tory tests revealed important leucocytosis and the ab­do­mi­nal ul­tra­sound highlighted a massive sple­no­me­ga­ly. He was hos­pi­ta­lized for further investi­ga­tions. We performed all the necessary laboratory tests to establish the diagnosis of the patient. Even though the osteomedullar biopsy and the flow cytometry suggested the diagnosis of acute B-cell lymphoblastic leukemia, the fluorescence in situ hybri­di­za­tion exam – the translocation t(9;22) was present in 100% of the analyzed cells – and the detection of BCR-ABL1 b2a2 trans­cript established the diagnosis of chronic myeloid leu­ke­mia, B-cell lymphoblastic crisis. We decided to start the treat­ment with the GRAAPH 2005 pro­to­col associated with ima­ti­nib, and the patient was a candidate for allogeneic trans­plan­ta­tion. We chose to pre­sent this case because the pa­tient was young, without sig­ni­fi­cant comorbidities, with chronic myeloid leukemia – B-cell lymphoblastic crisis as the initial diagnosis, whose evo­lu­tion was negative, despite his favorable prognosis.

Chronic myeloid leukemia presenting in lymphoblastic crisis, a differential diagnosis with Philadelphia-positive B-lymphoblastic leukemia

Rare patients with chronic myeloid leukemia (CML) can present initially in lymphoblastic crisis (LBC) mimicking Ph + B-lymphoblastic leukemia (B-ALL). We retrospectively reviewed 275 adults who diagnosed initially as Ph + B-ALL and identified 28 patients with at least one of three features supporting the diagnosis of CML-LBC: 1) a large discrepancy between the blast count and Ph + clone; 2) Ph + clone persistent when B-ALL in remission; 3) BCR/ABL1 fusion detected in segmented cells. BCR-ABL1 fusions were p210 in 25 patients and p190 in 3 patients. In comparison to patients with Ph + B-ALL, patients with CML-LBC were older; had higher leukocyte and absolute neutrophil counts; higher immature myeloid cells in peripheral blood; lower blast counts; and inferior outcomes. In addition, we prospectively analyzed 26 patients with Ph + B-lymphoblastic leukemia and identified 8 patients with features more consistent with CML-LBC. These findings highlight the importance of distinguishing CML-LBC from de novo Ph + B-ALL.

Clinical Features and Outcomes of Patients With Chronic Myeloid Leukemia Presenting With Isolated Thrombocytosis: A Systematic Review and a Case From Our Institution.

Chronic myeloid leukemia (CML) represents a common condition in the spectrum of myeloproliferative disorders (MPD). It classically exhibits leukocytosis, but rarely presents with isolated thrombocytosis. This paper is designed to review the clinicopathologic features, treatment, and outcomes of patients with CML who present with isolated thrombocytosis.We searched PubMed, MEDLINE®, ScienceDirect, and Scopus for English-language articles about case series and case reports for the period 2000-2020 with the terms “chronic myeloid leukemia” and “thrombocytosis” and pooled them with a case from our institution. Cases were also incorporated from the reference list and screened for inclusion. A total of 20 cases were included in the final cohort. The male-to-female ratio was 1:1.86. The mean age of the patients at the time of initial diagnosis was 40.5 years (range: 9-77 years). Out of 17 cases with available data, seven (41%) were asymptomatic and found to have thrombocytosis incidentally upon routine blood work. Five cases (29.4%) either had a history of thrombotic events or presented with severe thrombotic complications, including ischemic cerebrovascular accidents (CVA), myocardial infarction (MI), pulmonary embolism (PE), and/or miscarriages. Four cases (23.5%) had more than one symptom at presentation, including headache, syncope, and bruising. The average platelet count was 1,923 × 109/L (range: 584-8,688 × 109/L), and one case (5%) had anemia. The bone marrow (BM) examination showed normal cellularity and normal myeloid to erythroid (M/E) ratio in seven (50%) and 11 (84.6%) out of the 14 and 13 cases with reported data, respectively. Moreover, megakaryocytes in the BM were small in 10 cases (71.4%), pleomorphic in three cases (21.4%), and dysplastic in one case (7.1%).Accurate differentiation among MPD subtypes and the exclusion of CML is critical in reaching a proper diagnosis to decide on proper therapy and eventually modify outcomes. Prompt evaluation for the precise diagnosis of patients presenting with isolated marked thrombocytosis will help expedite their diagnosis and initiation of a specific tyrosine kinase inhibitor (TKI) therapy, thereby promptly inducing remission, preventing thrombotic complications, and avoiding adverse drug events, which would eventually improve outcomes.

Management of Priapism with Chronic Myeloid Lukaemia- A Rare Presentation and Our Experiences

Background: Ischaemic priapism is one of rare presentation of Chronic myeloid leukaemia due hyper viscosity and it needs urgent urological intervention. Objective: To evaluate the need of urological intervention in Priapism with Chronic Myeloid Leukaemia (CML). Methodology: This is a retrospective study from January 2014 to July 2019 in the department of Urology, DMCH. Among 126 patients 6 were diagnosed as Priapism with CML. Records of these patients were evaluated and analyzed. Results: Among 126 patients 6 patients were diagnosed as priapism with CML Mean age of the patient was 46.7 years, 2 of them were farmer and 2 had history of taking herbal drugs immediate before sexual intercourse and developed Priapism. Five patients were newly diagnosed as CML after admission in Urology ward. Of these six cases five were managed with minimal invasive procedure such as intermittent pseudo-ephedrine injection and wide bore needle corporal aspiration with pseudo-ephedrine irrigation as emergency management, only one needed surgery that was proximal shunt. After emergency management all patients were sent to hematologist for definite management of CML. Conclusion: Priapism with CML is a medical emergency which need immediate minimal intervention with concomitant haematological management.

Chronic myeloid leukemia presenting with isolated thrombocytosis: a report of two cases

孤立性血小板增高慢性粒细胞白血病（CML）是一类临床症状不典型的CML，易与原发性血小板增多症混淆。本文报道2例以孤立性血小板增高为表现的CML，通过巨核细胞形态、分子生物学及细胞遗传学检测等手段加以甄别。临床应重视这类变异型CML的存在，以免误诊误治。

Unusual Presentation of Chronic Myeloid Leukemia in Chronic Phase as Multiple Soft Tissue Chloromas.

Unusual Presentation of Chronic Myeloid Leukemia in Chronic Phase as Multiple Soft Tissue Chloromas.

Spontaneous Acute Subdural Hematoma as an Initial Presentation of Chronic Myeloid Leukemia.

Spontaneous Acute Subdural Hematoma as an Initial Presentation of Chronic Myeloid Leukemia.