Manifestation Of Acute Lymphoblastic Leukemia Research Articles

Bilateral Acute Proptosis With Papilledema and Sub-retinal Fluid As Initial Manifestations of Acute Lymphoblastic Leukemia in a Child

Bilateral Acute Proptosis With Papilledema and Sub-retinal Fluid As Initial Manifestations of Acute Lymphoblastic Leukemia in a Child

Vertebrae compression fracture due to osteonecrosis as initial sign of childhood acute lymphoblastic leukemia: A case report

BACKGROUND: Acute lymphoblastic leukemia (ALL) is the most common cancer in children. Manifestations of ALL vary from pallor, organomegaly, fever, and pancytopenia. Bone pain caused by vertebrae fracture is an uncommon manifestation of ALL, approximately only 16% of all bone pain occurrences. Fractures in ALL could occur at diagnosis and following treatment initiation. This case report described vertebrae compression fracture due to osteonecrosis as the initial sign of childhood acute lymphoblastic leukemia. CASE: An 8-year-10-month girl was referred to an orthopedic because of worsened low back pain since 1 month before, with no history of trauma. The pain was throbbing and radiated to the lower extremities. The patient was non-ambulant due to the pain. Physical examination revealed the visual analog scale was 8, tenderness in the spine, decreased strength, and range of movement in the lower extremities. There was no organomegaly in this patient. Serial laboratory examination revealed mild pancytopenia (leukopenia, mild normochromic normocytic anemia, mild thrombocytopenia) with dominancy of lymphocytes (68.1-81.9%). No blast was found in the peripheral blood smear. Magnetic Resonance Imaging of the spine revealed compression of the vertebral corpus with a pincer fracture and bone infarction. Bone marrow aspiration showed ALL-L2 with lymphoblast 50%. The patient started chemotherapy with ALL standard risk protocols and got zoledronic acid during the treatment. The patient's condition gradually improved, and able to undergo chemotherapy protocol. No side effects of zoledronic acid occurred. CONCLUSION: Vertebrae compression fracture without trauma and pancytopenia can be an early symptom of ALL. Zoledronic acid can be given concurrently with chemotherapy for bone mineralization.

Heart failure as a manifestation of acute lymphoblastic leukemia

Clinically significant myocardial infiltration by blast cells is a rare occurrence in acute leukemia. Heart failure rate in children with hemato-oncological diseases is unknown. Here we report a clinical case of an 8-year-old female patient with B-cell acute lymphoblastic leukemia as well as heart failure that resolved during specific therapy for the cancer. The patient's parents gave consent to the use of their child's data, including photographs, for research purposes and in publications.

Bilateral Malignant Pleural Effusion as an Initial Manifestation of Acute Lymphoblastic Leukemia: A Rare Case Report

Bilateral Malignant Pleural Effusion as an Initial Manifestation of Acute Lymphoblastic Leukemia: A Rare Case Report

Unilateral Coxalgia Revealing Acute Leukemia: One Case Report and Review of Clinical Literature

Introduction: The osteoarticular manifestations revealing acute leukemia in children are diverse and can be source of diagnostic delay, especially in the absence of other signs that point to a malignant origin. Case Report: We report through this case of a 4-year-old boy without any medical history and who consulted in our department for isolated severe pain in the left hip bone causing sleep deprivation, occurred after a minimal trauma and causing lameness, all symptoms dated back to 15 days before admission. Clinical examination was normal. Radiological assessment was normal as well. Mild anemia with neutropenia was noted on the blood count with no inflammatory signs. The blood smear was normal. The bone marrow aspiration confirmed the diagnosis of acute B-cell lymphoblastic leukemia. Discussion: Acute lymphoblastic leukemia (ALL) is the most common childhood malignancy. Osteoarticular manifestations of ALL in children are rare, they are revealing ALL in 14% of cases.The musculoskeletal symptoms can be so prominent or even the only presenting sign that sometimes the diagnosis of leukemia is delayed. Conclusion: Knowledge of orthopedic manifestation of leukemia is important in the diagnosis, supportive treatment and follow up of patients in order to improve their survival.

Osteoarticular manifestation of acute lymphoblastic leukemia in adults: a literature review.

Osteoarticular manifestations such as arthritis and bone pain are scarce among adults with acute lymphoblastic leukemia (ALL). We present a systematic review of osteoarticular first clinical manifestation related to ALL in adults, and we report a case of an adult patient with a B-cell ALL revealed by refractory pygalgia and arthritis. A systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guideline using the MEDLINE database, including case reports and case series describing osteoarticular manifestations revealing ALL in adults. There were 29 patients with osteoarticular manifestations, revealing ALL (including our case). The mean age was 34.00 ± 13.29years. Osteoarticular manifestations were peripheral articular signs (7 cases), axial manifestations (17 cases), and osteolytic lesions (21 cases). Vertebral fractures were reported in 4 cases. MRI was performed in 15 cases, showing heterogeneous signal changes in the vertebra, skull, and sacroiliac bones. It showed avascular necrosis of the femoral head in one case. PET scan, performed in 7 cases, showed diffuse or localized FDG uptakes in the bone marrow. Hypercalcemia was noted in 9 cases. The treatment was based on chemotherapy (23 patients) and radiotherapy (4 cases). During the follow-up, remission was noted in 14 cases, death in 9 cases, and was not available in 6 patients. Our review showed that axial manifestations, joint swelling, bone pain, and hypercalcemia could be the first and only symptoms of ALL in adults, making the diagnosis of ALL difficult to recognize, leading to a diagnosis delay. Key Points • Acute lymphoblastic leukemia in adults revealed by osteoarticular manifestations can be misdiagnosed as rheumatic diseases. •Axial manifestations, joint swelling, bone pain, and hypercalcemia could be the first and only symptoms of acute lymphoblastic leukemia in adults. • Complete blood count and calcium blood test should be performed as first-line investigations in adults with axial or peripheral articular symptoms. •Physicians should be aware of this clinical presentation to avoid diagnosis delay and improve prognosis.

Osteolytic Bone Lesions As an Initial Presenting Manifestation of Acute Lymphoblastic Leukemia in Adults

Introduction Hematological malignancies can lead to bone lesions, most commonly the osteolytic lesions found in multiple myeloma (MM). Cases of osteolytic lesions have been rarely reported in acute lymphoblastic leukemia (ALL), non-Hodgkin lymphoma, Waldenström macroglobulinemia, chronic lymphocytic leukemia (CLL), acute myeloid leukemia (AML), and myeloproliferative neoplasms (MPN). This review sheds light on the association between ALL and osteolytic bone lesions. Methods A literature search was performed for English language articles using PubMed, Scopus, and Google Scholar, for any date up to May 2022. The following keywords were used "ALL," "acute lymphoblastic leukemia," osteolytic bone lesions," and "osteolysis." Search results were initially reviewed by title and abstract, and articles were selected for more in-depth analysis if deemed relevant. Pertinent articles were examined in-depth for this report. Results To our knowledge, we found 15 cases of patients with ALL who developed osteolytic lesions. They were reported in 14 articles published between April 1998 and May 2022. Most patients were males (12 out of 15). The median age was 29 years (ranging from 22 to 78 years), with most patients being young (in the third and fourth decades). B-ALL was the most common type of ALL associated with osteolytic lesions (14 cases). All patients presented with bone pain, and hypercalcemia was found in 80% of the reported cases. Osteolytic lesions were detected by plain radiography(x-ray) in approximately half of the patients. The osteolytic lesions were confirmed in the remaining patients by CT, MRI, or PET scans. The most affected bones were the vertebrae, skull, and pelvis. The clinical outcomes among these patients were variable; two of the reported cases died after the diagnosis of osteolytic lesions. Discussion Bone metastases are classified as osteolytic, osteoblastic, or mixed based on the primary interference mechanism with bone remodeling. The destruction of normal bone characterizes osteolytic bone metastasis, occurring in multiple myeloma (MM), renal cell carcinoma, melanoma, non-small cell lung cancer, non-Hodgkin lymphoma, thyroid cancer, or Langerhans-cell histiocytosis. Osteolytic metastasis in adults with ALL is an extremely rare condition. The exact pathophysiology of osteolysis in ALL patients is unknown.PTHrP secretion by lymphoblasts has been shown to be the primary cause of osteolysis in ALL patients. Additionally, serum from ALL patients with osteolysis can induce in vitro proliferation of osteoclasts, indicating the mechanism of lymphoblasts induces osteoclasts activation in vivo. Based on our review, there was no association between osteolytic bone lesions and the Philadelphia chromosome detected in only one patient. Osteolytic bone lesions in ALL patients mainly present with bone pain. Pathological fracture of the vertebral body leading to spinal cord compression is a complication of osteolytic lesions in patients with MM. However, there is no case of spinal cord compression in ALL patients attributed to osteolytic lesions of the vertebra. Given that our review is based on case reports, we could not conclude if the presence of osteolytic bone lesions is a prognostic factor for adverse outcomes or indicates an "aggressive" form of leukemia in adult patients with ALL. Conclusion Osteolytic bone lesions in adult patients with ALL is an infrequent finding and mainly occurs in young adults. Plain radiography is often sufficient in diagnosing osteolytic bone lesions; however, other imaging modalities are sometimes required. Based on our review, the osteolytic lesions do not indicate a poor prognosis in adult patients with ALL. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal

Recurrence of Lymphoblastic Leukemia Manifesting as Bilateral Optic Disk Neovascularization During Complete Clinical Remission--Case Report

<i>Background</i>: The intraocular manifestations of leukemia are diverse. The retina is the most common ophthalmic involvement site. The most common retinal manifestations of acute lymphoblastic leukemia (ALL) include dilated and tortuous vessels, serous retinal detachment (SRD), intraretinal hemorrhages as well as roth spots. Optic nerve infiltration and retinal neovascularization are unusual, especially in complete clinical remission of ALL. However, neovascularization in optic disk has not been observed in ALL during complete clinical remission. <i>Objective</i>: To report clinical characteristics of a bilateral optic disk neovascularization associated with relapse of ALL. <i>Method</i>: A 31-year-old male with ALL after having reached complete remission from chemotherapy complained about a vision loss in both eyes. First examination showed that best corrected visual acuity (BCVA) was 20/200 for his right eye and 20/160 for his left eye, respectively. Fundus examination revealed bilateral profound optic disk edema with multiple yellow-white exudates and retinal hemorrhages. Optical coherence tomography (OCT) showed bilateral optic disk edema and SRD in both maculas. Fundus fluorescein angiography (FFA) showed bilateral optic disk neovascularization. All these changes were present in remission. The patient received intravitreal Conbercept injection 0.5 mg (10 mg/ml) in both eyes for once on June 29, 2019. Optic disk neovascularization resolved three weeks after treatment. BCVA improved to 20/200 in his right eye, but still light perception in his right eye. No optic disk neovascularization recurrence was observed in 3-month follow-up. BCVA of the right eye maintained 20/200 at the latest follow-up. After intravitreal injection for 4 months the patient suffered from cerebral hemorrhage. He received bone marrow biopsy and cerebrospinal fluid sampling. The results showed relapse in hemogram and lymphocytes in central nervous system (CNS). Therefore, the chemotherapy treatment did not cause a new remission of the disease and the patient failed to recover. <i>Conclusion</i>: Optic disk neovascularization may appear as a sign of extramedullary relapse of ALL months in advance of the hematologic relapse. Intravitreal injection of conbercept could be beneficial and safe in treating this neovascularization.

Severe Hypercalcemia as Rare Manifestation of Acute Lymphoblastic Leukemia in Adolescent

Background: Hypercalcemia is a rare manifestation of acute lymphoblastic leukemia (ALL). Several studies reported that severe hypercalcemia is very uncommon in pediatric ALL, but there is no report regarding ALL in adolescence and young adult (AYA) which comprises distinct entity with diverse prognosis. Clinical Case: A 18-year-old male presented with prolonged fever, general weakness, fatigue, pale and decrease of body weight for 3 months. He also complained visual disturbances which progressively worsened for 1 month. On admission, systemic examination showed that he was alert, had tachycardia, proptosis of both eyes and splenomegaly. There was neither lymphadenopathy nor thyroid/parathyroid abnormality. Initial laboratory investigation revealed pancytopenia (hemoglobin of 6.8 g/dl, hematocrit of 19.1%, white blood cell count of 3.8 x 109/L with platelets of 58 x 109/L and no blast). There was severe hypercalcemia of 18 mg/dL, hypomagnesemia 1.14 mg/dL, with normal phosphorus level. His serum creatinine level slightly increased to 1.3 mg/dL with filtration rate of 79 ml/min/1.73 m2. Bone marrow evaluation showed blast cells infiltration, consistent with ALL type L3. Leukemia phenotyping revealed B cell-lineage with aberrant exp CD5. CT scan orbita and brain showed bilateral retrobulbar mass and diffuse lytic lesion in skeletal bones due to leukemic cells infiltration. Patient was treated with supplemental oxygen, hydration targeting natriuresis, loop diuretic and steroid. The serum calcium level remained high, thus bisphosphonate was given. There was progressive decline of serum calcium level after therapy. The patient is then prepared to start chemotherapy. Discussion: Hypercalcemia is associated with both PTH or PTH-independent mechanism, including malignancy. It complicates 5–20% of malignancy, but only 0.6–4.8% reported in leukemia. Hypercalcemia in malignancy may present due to: (1) direct invasion of cancer cells to bone and (2) secretion of humoral PTH-related peptide (PTHrP) which mimics PTH. Severe hypercalcemia without inadequate treatment is fatal. Initial treatment of hypercalcemia includes hydration, loop diuretic, calcitonin and bisphosphonate targeting decline of serum calcium level of 3–9 mg/dL in 24–48 hours. The main treatment of leukemia-related hypercalcemia is chemotherapy. Several cases of pediatric ALL reported normocalcemia after starting induction phase of chemotherapy and exerted good prognosis. Conclusion: Hypercalcemia should be investigated in leukemia patients and vice versa. The main aim of therapy is to normalize the serum calcium level and treat underlying causes. In adolescent ALL, adequate treatment of hypercalcemia helps achieving better outcome.

Safety and Efficacy of CD19 CAR T-Cells for Pediatric Relapsed Acute Lymphoblastic Leukemia with Active CNS Involvement

Background: CAR T cells targeting CD19 are approved for patients with relapsed or refractory acute lymphoblastic leukemia (ALL), failing two or more prior therapies. The central nervous system (CNS) is a known sanctuary site of ALL. Despite observations of CAR T-cells trafficking to the CNS in patients, most clinical trials excluded patients with active CNS leukemia, partially for concerns of neurotoxicity. Methods: We conducted an international retrospective study of patients who were referred to CAR T-cell therapy for relapsed ALL with CNS involvement. Results: We reviewed data of 27 patients (16 males and 11 females) from 8 centers who were treated with CD19 CAR T-cells for ALL relapse involving the CNS. Fifteen patients had a prior bone marrow transplantation, and in seven total body irradiation was given. Four additional patients received previous cranial radiation. At time of CAR T-cell order or clinical trial inclusion, ten patients had an isolated CNS relapse, and 17 had a combined CNS and marrow relapse. The median number of cells in the CSF of patients at enrollment was 65 (range 0-5670), and 16 of 21 patients who had imaging performed showed leukemia-associated changes in brain MRI. All patients received bridging chemotherapy between enrolment and lymphodepletion, including intrathecal chemotherapy alone or in combination with systemic chemotherapy and/or radiation. At lymphodepletion, 15 patients had cleared CNS disease while 12 had active CNS blasts or imaging lesions. Following lymphodepletion with fludarabine and cyclophosphamide, patients received 2nd-generation CAR T cells: 15 patients treated with a CD19-4-1BB CAR and 12 with a CD19-CD28 CAR. Cytokine release syndrome (CRS) occurred in 20 patients: 12 patients (100%) treated with CD19-CD28 CARs and 8 with CD19-4-1BB CARs (53%, p=0.006). Immune-effector cell neurotoxicity (ICANS) was diagnosed in 11 patients, 9 of 12 following CD28-based CARs, and 3 of 15 following 41BB-based CARs (p=0.004). Patients with active CNS-disease prior to lymphodepletion also had a higher rate of ICANS (75% vs 20%, p=0.004) but not of CRS. One patient died of grade 5 ICANS on day +7 following CD28-based CARs for an isolated CNS relapse. Twenty-four of 26 evaluable patients (92%) had a complete remission following CAR T-cells. Nine patients were further referred to an allogeneic transplant, all following CD28-based CARs. Overall, six patients had relapsed - in four relapse involved bone marrow, and in two the CNS. Conclusions: In this cohort of patients with active CNS disease treated with CD19 CAR T-cells, we report a high remission rate in previously heavily pre-treated patients. Toxicities are significant, and associated mostly with the costimulatory domain of the CAR (CD28&amp;gt;41BB) and the presence of active CNS disease at lymphodepletion. This may be informative when designing future clinical studies of CAR T cell therapy for patients with CNS manifestations of ALL. Disclosures Jacoby: Novartis: Honoraria, Membership on an entity's Board of Directors or advisory committees; Lonza: Membership on an entity's Board of Directors or advisory committees. Ghorashian:Novartis: Honoraria; UCLB: Patents &amp; Royalties; Amgen: Honoraria. De Moerloose:Novartis: Consultancy. Rossig:Celgene: Membership on an entity's Board of Directors or advisory committees; Novartis: Membership on an entity's Board of Directors or advisory committees; Roche: Honoraria, Membership on an entity's Board of Directors or advisory committees; Amgen: Membership on an entity's Board of Directors or advisory committees; BMS: Honoraria; EUSA Pharma: Membership on an entity's Board of Directors or advisory committees; Genetech: Membership on an entity's Board of Directors or advisory committees; Pfizer: Honoraria. von Stackelberg:Amgen: Membership on an entity's Board of Directors or advisory committees, Other: Personal fees, advisory committees and speakers bureau, Speakers Bureau; Jazz: Membership on an entity's Board of Directors or advisory committees, Other: Personal fees, advisory committees and speakers bureau, Speakers Bureau; Roche: Membership on an entity's Board of Directors or advisory committees, Other: Personal fees, advisory committees and speakers bureau, Speakers Bureau; Morphosys: Membership on an entity's Board of Directors or advisory committees, Other: Personal fees, advisory committees and speakers bureau, Speakers Bureau; Shire: Membership on an entity's Board of Directors or advisory committees, Other: Personal fees, advisory committees and speakers bureau, Speakers Bureau. Bourquin:Servier: Other: Travel Support. OffLabel Disclosure: We describe the use of commercial and investigational CAR T-cell products

Случай развития переднего увеита на фоне острого лимфобластного лейкоза

A clinical case of the development of a rare form of ocular manifestation of acute lymphoblastic leukemia, one of the most common oncological diseases in childhood, is described. A seven-year-old child initially had weakness, decreased appetite, and a single episode of subfebrile condition for two days without catarrhal symptoms. Further, an increase in the cervical lymph nodes was noticed. Then a hemorrhagic rash appeared all over the body. In the complete blood count (CBC) test: erythrocytes (RBC) – 3.8 * 1012 / l, hemoglobin (HGB) – 115 g/L, leukocytes (WBC) – 121*109/L, platelets (PLT) – 22*109/L. The child was admitted to the regional children’s clinical hospital. On examination, 90% of blast cells were detected in the myelogram. Against the background of a confirmed remission of the oncological disease, iridocyclitis of the left eye was diagnosed. It could not be stopped by standard antibacterial and antiinflammatory therapy. After a while, the right eye was also involved in the pathological process. Hematologists confirmed the recurrence of acute lymphoblastic leukemia after bone marrow puncture. The presented clinical case demonstrates the fact that the treatment of ocular manifestations of acute lymphoblastic leukemia can be effective only in the treatment of the underlying disease.

Leukocytoclastic vasculitis as a previously unreported paraneoplastic manifestation of acute lymphoblastic leukemia in adults

Although leukocytoclastic vasculitis (LCV) is often idiopathic or secondary to benign diseases, it sometimes accompanies malignancies. Besides being more common in hematological cancers compared to solid tumors, the association of acute lymphoblastic leukemia (ALL) with LCV is extremely rare. In this study, we present a 22-year-old woman presenting with palpable petechiae, purpura, and ecchymoses on her limbs. Bone marrow and skin biopsy of the patient with bicytopenia revealed precursor B cell ALL and LCV, respectively. This case is the first adult ALL case with paraneoplastic LCV. In this study, it is emphasized that hematological malignancies should be considered in patients presenting with cutaneous vasculitis, especially in the presence of cytopenia.

Rapid Weight Gain: An Unusual Presentation of Leukemia

AbstractNeurological manifestations of leukemia can be due to direct effects of the malignancy or due to the indirect effects of infection or therapy. An 11-year-old boy presented with recent-onset weight gain with papilledema and a history of tuberculosis contact. Neuroimaging and initial microscopic examination of the cerebrospinal fluid (CSF) did not aid in the diagnosis of central nervous system leukemia. He was started on antitubercular treatment yet deteriorated. Repeat CSF analysis when subjected to cytospin and flow cytometry confirmed the diagnosis of B cell acute lymphoblastic leukemia (ALL). Although there have been reports of relapsed ALL presenting as obesity, isolated rapid changes in weight at initial presentation are a very rare and unusual manifestation of ALL. To the best of our knowledge, this is the first such report.

Non-oliguric renal failure with nephromegaly as the presenting manifestation of acute lymphoblastic leukemia in a young adult: An unusual case report

Non-oliguric renal failure with nephromegaly as the presenting manifestation of acute lymphoblastic leukemia in a young adult: An unusual case report

On importance of correct interpretation of complete blood count

We would like to present the case the manifestation of acute lymphoblastic leukemia in 2-year-old and 11-month child was treated with antiviral therapy during several month. We retrospectively analyzed hemogram’s values of the child and the importance of correct and timely interpretation of complete blood count is once again evidently demonstrated.

Life-threatening Hypercalcemia as the First Manifestation of Acute Lymphoblastic Leukemia

Hypercalcemia of malignancy, usually reported in adults in advanced stages, is rare in children. A 4-year-old boy presented with intermittent episodes of severe hypercalcemia, which improved with intravenous hydration therapy, furosemide and bisphosphonates as the initial manifestation of occult acute lymphoblastic leukemia. Pediatricians should rule out hematological malignancy in patients with severe hypercalcemia.

Exudative retinal detachment as the sole manifestation of acute lymphoblastic leukemia

The uterine effects of polyethylene IUDs were studied in 11 adult female rabbits. 9 implantation sites were observed in the IUD-containing uterine horns, while 39 were observed in contralateral control horns. The implants were located between the ovary and IUD, and the resorptions were found close to the IUD. The white blood cell count of uterine fluid in the IUD horn was about twice that in the control uterine horn, while uterine fluid micro-pH values were slightly lower in IUD-containing horns than in controls. IUD-containing horns had about 50% of the stainable material that controls had. The results suggest that the lytic enzyme freed from the uterine mucosa is insufficient to effect a maximum antifertility response in polyethylene IUD-treated rabbits.

Unusual Presentations of Acute Lymphoblastic Leukemia in Children: A Study of 9 Patients

Background: Acute lymphoblastic leukemia is the most common malignancy in children below 15 years of age. Bone marrow, CNS and the gonads are the three major sites of failure or relapse in acute lymphoblastic leukemia. Occasionally, leukemic cells can infiltrate the unusual sites and present with some unusual manifestations either at initial (diagnosis) presentation or during the course of disease. This study presents 9 such rare cases of unusual manifestations of acute lymphoblastic leukemia. Aim: To report the unusual presentation and management of acute lymphatic leukemia in children either at initial diagnosis or during the course of disease. Methods: Between 2000-2012, 1800 patients with acute lymphoblastic leukemia were treated at the Gujarat Cancer &amp; Research Institute, Ahmedabad, a tertiary cancer care center and one of the largest regional cancer center of India. Among these patients a total of 317 cases were found to develop relapse at bone marrow, CNS, testicles etc. Out of these patients, a total of 9 patients were detected to have very unusual manifestations of the disease, either at initial presentation or during the course of their disease. These patients had parotid salivary gland involvement (1), maxillary sinus (2), bilateral blindness due to retinal detachment (1), bilateral facial nerve palsies (1), multiple symmetrical involvement of bones of both upper and lower extremities (1), small bone of the hand (4th metacarpal bone) (1), massive splenomegaly (1) and torsion of the testicle (1). The manifestations of such unusual clinical presentations, their evaluation, management and ultimate outcome is being presented in this presentation along with review of the literature. Results: Acute lymphoblastic leukemia can present with very unusual manifestation during initial diagnosis or later. Conclusion: ALL may occasionally present in some unusual way. Such uncommon presentation can create a diagnostic dilemma as well as difficulty in management. All such presentations require special attention from the treating consultant and team for the better outcome. We strongly recommend that all such rare presentations must be reported to enhance the understanding of the disease and enhance the literature.

Clinico-epidemiological study of extramedullary disease manifestations in childhood acute lymphoblastic leukaemia in a tertiary care centre

Background: Data on extramedullary manifestation of acute lymphoblastic leukemia (ALL) in Indian children are very limited, authors analyzed the clinical, epidemiological and immunophenotype profile of these manifestations in our pediatric patients.Methods: This was a prospective observational study in which data of 46 children with ALL, aged 0 to 14 years were analysed.Results: Among the total 46 patients of ALL admitted 86.95% presented with extra medullary features. Definite male preponderance (67.5%) was observed, 80% patients were aged between one to ten years. Common extra medullary features included hepatomegaly (95%), followed by splenomegaly (77%), lymphadenopathy (58%), arthritis (17.5%), and CNS involvement in 5%. Mediastinal mass was seen in 5 %. Majority of them (67.5%) had White blood cell (WBC) count less than 20,000/mcL. 92.5% were Pre B ALL, while T ALL was seen in three (7.5%) patients. Out of these three T cell ALL two presented with mediastinal mass and one with CNS leukemia.Conclusions: The clinicians must be aware of the constellation of different extramedullary features to diagnose ALL. Early diagnosis and prompt treatment may improve the clinical outcome in these patients. Authors suggest larger multi-centric studies to understand the clinical and laboratory profile of extramedullary disease in ALL for better risk stratification to help in risk-adopted therapy.

Trismus as an Orofacial Manifestation of Acute Lymphoblastic Leukemia

Trismus as an Orofacial Manifestation of Acute Lymphoblastic Leukemia