Erythroleukemia: a clinco-hematological review of four cases.
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Hepatosplenomegaly
Pallor
Bone marrow examination
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Myeloid sarcoma (MS) is the tumor of immature myeloid cells involving extramedullary sites. Liver involvement of MS is rare in infant. Three months old female infant presented with hepatosplenomegaly and bicytopenia. Repeated bone marrow aspiration detected no blasts and flow cytometric analysis was normal. Abdominal magnetic resonance imaging revealed multiple nodular lesions in the liver. The biopsy results were consistent with MS. She presented with paleness and fever 8 months later. She had ongoing deep anemia, thrombocytopenia and hepatosplenomegaly. Bone marrow biopsy showed blast cell infiltration with 20% cells positive for CD61. The bone marrow karyotype investigation revealed complex quadruplet-translocation with the 46, X, t(X; 11; 22; 1) [15]/46, XX [1] karyotype. AML M7 was diagnosed and chemotherapy started. MS may occur as initial manifestation of AML with t(1;22) which is often associated with marrow fibrosis making sampling difficult. Hence cytogenetic analysis is of paramount importance in making an accurate diagnosis.
Hepatosplenomegaly
Myeloid sarcoma
Acute megakaryoblastic leukemia
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The association of plasmacytosis and lymphocytosis with acute myeloid leukemia (AML) has been documented in isolated case reports. We examined 149 cases (134 adults, 15 children) of newly diagnosed AML and found 9 adults (6%) with > or = 5% plasma cells and 1 child and 1 adult with > or = 20% lymphocytes. Lymphocytes constituted 25% and 42% of marrow cellularity in the adult and child respectively and persisted throughout remission in the child's marrow. The percentage of morphologically normal plasma cells ranged from 5% to 13% (mean 7%). Monoclonal immunoglobulins were not detected with immunostaining or flow cytometry. Hypergammaglobulinemia was present in 3 cases, and a monoclonal increase in IgG-kappa in 1. Plasmacytosis was not seen in remission marrows from these patients (n = 4). Lymphocytosis or plasmacytosis occurs in approximately 7% of patients with AML, appears reactive in nature, and may represent an immunological response to tumor. Monoclonal paraproteins may occur without other evidence of B-cell neoplasia.
Plasmacytosis
Lymphocytosis
Paraproteins
Acute lymphocytic leukemia
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Patients suffering from adult acute lymphoblastic leukemia are acutely ill and present most commonly with fever, pallor, bleeding, lymphadenopathy, hepatosplenomegaly and presence of lymphoblasts in the peripheral blood and bone marrow. We describe a rare presentation of acute lymphoblastic leukemia, in a young adult male who had vague and minimal symptoms with mild splenomegaly. There was severe eosinophilia along with absence of blasts in the peripheral blood, and 40% blasts with increase in eosinophils in the bone marrow. The blasts were positive for common precursor B cell markers on flow cytometry. The patient had a unique cytogenetic abnormality t(7;12)(q22;p13),-9, not previously described in acute lymphoblastic leukemia. He was categorized as poor risk due to failure to achieve complete remission after induction with UK ALL XII chemotherapy.
Hepatosplenomegaly
Hematology
Pallor
Hematopathology
Immunophenotyping
Lymphoblast
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An unusual cutaneous tumor that has blastic morphology and coexpresses CD56 and terminal deoxynucleotidyl transferase (TdT) has been recently recognized and termed blastic natural killer cell lymphoma.The authors identified seven cases of such CD56(+)TdT(+) blastic tumors presenting in skin at their institution. The authors correlated clinical course with histomorphology and immunophenotype.All 7 patients (6 men, 1 woman, 52-85 years) presented with rapidly growing, frequently multiple cutaneous nodules. All patients had low level bone marrow involvement at diagnosis and frequently had lymph node involvement. Tumor cells were of intermediate size with irregular nuclear contours, fine chromatin, and indistinct small nucleoli. The expression of TdT varied between 5% and over 90% of the neoplastic cell population. Tumor cells were negative for surface CD3, CD5, and CD20 in all cases, but some patients showed expression of CD2 (three out of five), cytoplasmic CD3 (two out of seven), CD4 (six out of seven), and CD16 (three out of seven). Molecular studies showed absence of T-cell receptor gene rearrangements in all cases. All seven patients had rapid progression of disease, and six patients have died of their disease or complications. Three patients developed progressively increasing numbers of bone marrow blasts that had a myeloid immunophenotype and were negative for TdT and CD56. Two patients met criteria for acute myeloid leukemia at 11 and 22 months after presentation, respectively.CD56(+) TdT(+) blastic tumor presenting in skin is a systemic malignancy likely of primitive/undifferentiated hematopoietic origin. Patients might subsequently develop tumors of myeloid or myelomonocytic phenotype, indistinguishable from acute myelogenous leukemia.
Immunophenotyping
Terminal deoxynucleotidyl transferase
CD5
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It's a rare case of acute biphenotypic leukemia which initially presented in an elderly lady with generalized lymphadenopathy and mild hepatosplenomegaly. Biopsy of cervical lymph node was done and diagnosed as Non-Hodgkin's Lymphoma. Bone marrow smears showed 40% lymphoma cells, a few showing single large nucleolus. Peripheral blood smear showed moderate anemia and leucopenia but normal platelet count. On the basis of cervical biopsy and bone marrow findings, patient was given two cycles of Cyclophosphamide Hydroxydaurorubicin Oncoverin Prednisolone (CHOP) but she did not respond. In view of non responsiveness to CHOP, Immunophenotyping of bone marrow aspirate was done and result showed co expression of both T-lymphoid and myeloid markers in 90% of atypical cells along with marker of immaturity (CD34) in 72% cells. Analysis showed expression of CyCD3 in 90% cells, CD33 in 95% cells and 60% cells were cytochemically positive for Myeloperoxidase (MPO); thus it was a case of Biphenotypic Acute Leukemia (T-ALL+AML) as per WHO criteria for diagnosis of BAL. So it was an initial presentation of BAL with generalized lymphadenopathy and aleukemic peripheral picture.
Immunophenotyping
Generalized lymphadenopathy
Cervical lymphadenopathy
Lymph node biopsy
Hepatosplenomegaly
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Chronic myelomonocytic leukemia
Hematology
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A case of leukemia cutis (LC) of monocytic lineage in a patient with myelodysplastic syndrome (MDS) is presented. Cutaneous infiltrates were recognized concurrent with diagnosis of refractory anemia (RA) with monocytosis. Skin infiltrates subsequently spontaneously regressed although MDS progressed with increasing monocytosis, anemia, and thrombocytopenia. Death occurred 6 months after diagnosis with evolution of acute monoblastic leukemia complicated by sepsis. This case supports previous observations of poor prognosis associated with leukemia cutis. LC associated with MDS is reviewed including the role of monocytes. Am. J. Hematol. 66:120–122, 2001. © 2001 Wiley-Liss, Inc.
Monocytosis
Leukemia cutis
Acute monocytic leukemia
Cutis
Aplastic anemia
Refractory (planetary science)
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Acute erythroid leukemia (AML M6) is a hematopoietic neoplasm frequently described in cats and mice, rarely in other animal species. This report describes a case of AML M6 in a yearling Thoroughbred filly. Clinically the horse presented marked pale mucous membranes and exercise intolerance. In addition, the owner and referring veterinarian reported a 30-day history of progressive weight loss. The CBC revealed severe anemia and leukopenia by neutropenia. Cytology evaluation obtained from bone marrow fine needle aspirates evidenced inversion of the myeloid: erythroid ratio (0.2), with 48% of the nucleated cells corresponding to rubriblasts. In addition to the gross evidence of anemia, necropsy findings consisted of splenomegaly and lymphadenomegaly. The diagnosis of AML M6B was confirmed histologically due to splenomegaly and lymphadenomegaly, secondary to neoplastic metastasization.
Leukopenia
Preleukemia
Aplastic anemia
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Trisomy 10 as the sole cytogenetic abnormality in AML is rare, with an incidence rate of < 0.5%. It tends to affect the elderly and is extremely rare in pediatric patients. We describe a case of an 8-month-old Caucasian baby who presented with prominence of left eye and fever without lymphadenopathy or hepatosplenomegaly. Bone survey showed diffuse periosteal reaction in the femur, pelvis, maxillary and orbital bones (with fracture). CBC revealed normal white blood cell count with increased blasts, mild anemia and moderate thrombocytopenia. Bone marrow biopsy showed increased myeloblasts with bilineage dysplasia and 3-4+ reticulin fibrosis. Flow cytometry revealed blasts positive for CD34, CD33, and MPO and negative for CD7, CD13, and HLA-DR. Trisomy 10 was demonstrated by chromosome analysis and fluorescence in-situ hybridization. The patient received induction chemotherapy and achieved complete clinical and hematologic remission at day 28. However, he relapsed after three cycles of chemotherapy. Compared to the two other reported pediatric cases, our patient has some unique features such as much younger age and additional findings such as bilineage dysplasia and bone marrow fibrosis. Both reported cases and our case were classified as AML-M2 indicating that this may be a common subtype in pediatric patients. Bone involvement was present in our patient and one other case and both had similar immunophenotype (CD33+, CD7-). These findings suggest that isolated trisomy 10 may be associated with distinct clinicopathologic features in pediatric AML. Studies on additional patients are needed to establish this association.
Immunophenotyping
Hepatosplenomegaly
Trisomy 8
Trisomy
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Hemophagocytic Lymphohistiocytosis
Hematology
Human genetics
Lymphoproliferative Disorders
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