High-risk acute promyelocytic leukemia with unusual T/myeloid immunophenotype successfully treated with ATRA and arsenic trioxide-based regimen
We describe two patients with acute promyelocytic leukemia (APL) with an unusual immunophenotype with co-expression of myeloperoxidase (MPO) with cytoplasmic CD3 (cCD3) representing myeloid and T-lineage differentiation. Both harboredFLT3-ITD mutations. One additionally had a deletion in thePML gene affecting the primer binding site, thus limiting measurable residual disease (MRD) analysis during follow-up. Both patients achieved durable remission with all-trans retinoic acid (ATRA) and arsenic trioxide (ATO)-based therapy, thus mitigating the need for repetitive conventional chemotherapy cycles and allogeneic stem cell tr...
Source: Journal of Hematopathology - September 1, 2018 Category: Pathology Source Type: research

Adult T cell leukemia/lymphoma complicated by proliferation of large B cells: a diagnostic dilemma
AbstractPeripheral T cell lymphomas are clinically and pathologically complex and generally associated with overall poor prognosis and aggressive clinical course. In recent years, there is a greater recognition of abnormal B cell expansion as a component of T cell lymphomas especially those derived from follicular helper T cells. Most of these B cells are EBV positive and show a wide range of morphology which includes large mononuclear cells and Hodgkin-like cells. The number of the abnormal B cells can also vary. It is possible to misdiagnose this entity as a B cell lymphoma, Hodgkin lymphoma, composite lymphoma, or react...
Source: Journal of Hematopathology - September 1, 2018 Category: Pathology Source Type: research

Immunophenotypic and cytogenetic evolution patterns of the neoplastic plasma cells in multiple myeloma relapsed after stem cell transplant
AbstractMultiple myeloma (MM) is a neoplasm characterized by proliferation of clonal plasma cells (PCs) and a combination of clinical manifestations. Flow cytometry is an important method for diagnosing and monitoring of MM. Cytogenetic profiling of neoplastic PCs provides important prognostic information. Although stem cell transplantation (SCT) has significantly improved the overall survival of patients with MM, most SCT recipients relapse. We have studied the immunophenotypic and cytogenetic dissimilarities in the neoplastic PCs before SCT and after relapse in patients with initial complete remission, and investigated a...
Source: Journal of Hematopathology - September 1, 2018 Category: Pathology Source Type: research

Unusual morphology of multiple myeloma
AbstractMultiple myeloma can have plasma cells with unusual morphology and a high index of suspicion is required to diagnose multiple myeloma with atypical morphology. (Source: Journal of Hematopathology)
Source: Journal of Hematopathology - September 1, 2018 Category: Pathology Source Type: research

Immunophenotypic and cytogenetic evolution patterns of the neoplastic plasma cells in multiple myeloma relapsed after stem cell transplant
AbstractMultiple myeloma (MM) is a neoplasm characterized by proliferation of clonal plasma cells (PCs) and a combination of clinical manifestations. Flow cytometry is an important method for diagnosing and monitoring of MM. Cytogenetic profiling of neoplastic PCs provides important prognostic information. Although stem cell transplantation (SCT) has significantly improved the overall survival of patients with MM, most SCT recipients relapse. We have studied the immunophenotypic and cytogenetic dissimilarities in the neoplastic PCs before SCT and after relapse in patients with initial complete remission, and investigated a...
Source: Journal of Hematopathology - August 15, 2018 Category: Pathology Source Type: research

High-risk acute promyelocytic leukemia with unusual T/myeloid immunophenotype successfully treated with ATRA and arsenic trioxide-based regimen
We describe two patients with acute promyelocytic leukemia (APL) with an unusual immunophenotype with co-expression of myeloperoxidase (MPO) with cytoplasmic CD3 (cCD3) representing myeloid and T-lineage differentiation. Both harboredFLT3-ITD mutations. One additionally had a deletion in thePML gene affecting the primer binding site, thus limiting measurable residual disease (MRD) analysis during follow-up. Both patients achieved durable remission with all-trans retinoic acid (ATRA) and arsenic trioxide (ATO)-based therapy, thus mitigating the need for repetitive conventional chemotherapy cycles and allogeneic stem cell tr...
Source: Journal of Hematopathology - August 9, 2018 Category: Pathology Source Type: research

A unique case of mixed phenotype acute leukemia with t(9;22)(q34.1;q11.2); BCR-ABL1 sarcoma with epitheliotropism mimicking intestinal T cell lymphoma
We present an unusual case of myeloid sarcoma of T/myelomonocytic biphenotypic nature andBCR-ABL1 translocation demonstrating an epitheliotropism in the gastrointestinal tract and mass-forming lesions in the lungs without bone marrow involvement. A 69-year-old gentleman presented with non-bloody diarrhea and abdominal pain complicated by weight loss. CT scan showed diffuse mural thickening in the jejunum, distal ileum, transverse colon, and splenic flexure and bilateral pulmonary nodules. Biopsies of the ileum and colon were performed. An initial diagnosis of T cell lymphoma was made based on intestinal infiltration with C...
Source: Journal of Hematopathology - July 30, 2018 Category: Pathology Source Type: research

Adult T cell leukemia/lymphoma complicated by proliferation of large B cells: a diagnostic dilemma
AbstractPeripheral T cell lymphomas are clinically and pathologically complex and generally associated with overall poor prognosis and aggressive clinical course. In recent years, there is a greater recognition of abnormal B cell expansion as a component of T cell lymphomas especially those derived from follicular helper T cells. Most of these B cells are EBV positive and show a wide range of morphology which includes large mononuclear cells and Hodgkin-like cells. The number of the abnormal B cells can also vary. It is possible to misdiagnose this entity as a B cell lymphoma, Hodgkin lymphoma, composite lymphoma, or react...
Source: Journal of Hematopathology - July 18, 2018 Category: Pathology Source Type: research

Disseminated BCG-osis with haemophagocytosis, tubercular bacteraemia, and unusual haematological findings with its haematology analyser-based expression
AbstractInfantile disseminated BCG-osis is an uncommon complication of BCG vaccination and the presence of haemophagocytic lymphohistiocytosis (HLH) further complicates the clinical course due to its fatal outcome. Here, we describe a rare case of disseminated BCG-osis with HLH in a 3-month-old male child and the unusual morphological findings in the peripheral blood with its haematology analyser-based expression. The child presented with fever, failure to thrive, hepatosplenomegaly, erythematous skin rashes, and left axillary lymphadenopathy with history of BCG vaccination at birth. He was the first born of second-degree ...
Source: Journal of Hematopathology - July 15, 2018 Category: Pathology Source Type: research

Mutation of the DNMT3A and IDH1/2 genes in Iranian acute myeloid leukemia patients with normal karyotype (CN-AML): association with other gene mutation and clinical and laboratory characteristics
This study was purposed to investigate the frequency of DNMT3A and IDH1/2 gene mutation and the clinical features of Iranian cytogenetically normal acute myeloid leukemia (CN-AML) patients harboring these mutations. Thirty-nine CN-AML patients were recruited at the time of diagnosis. PCR followed by direct sequencing was used to detect the mutations of DNMT3A (R882), IDH1 (R132), and IDH2 (R140 and R172). The results showed that of all CN-AML patients, DNMT3A, IDH1, and IDH2 mutations were observed in five (12.8%), five (12.8%), and five (13.2%) patients, respectively. In addition, the most frequent DNMT3A, IDH1, and IDH2 ...
Source: Journal of Hematopathology - June 1, 2018 Category: Pathology Source Type: research

Leukemic phase of primary cutaneous anaplastic large-cell lymphoma (ALK-negative), with downregulation of CD30
We present a case of primary cutaneous-ALCL (C-ALCL), who developed nodal involvement 4  years later. The patient was treated with combination chemotherapy and autologous stem cell transplantation; however, she relapsed 5 months later. At that time, the patient was given brentuximab vedotin (BV) with initial response. One year later, the patient demonstrated progressive adenopathy an d leukemic involvement by her ALCL (comparative T cell clonality showed PCR products of the same size in the diagnostic skin and relapsed leukemic blood). Interestingly, the circulating tumor cells showed only subset expression of CD30 by fl...
Source: Journal of Hematopathology - June 1, 2018 Category: Pathology Source Type: research

Study of platelet activation markers and plasma cytokines in sickle cell disease patients during vaso-occlusive pain crises
AbstractThe present study was designed to examine platelet function during the sickle cell disease (SCD) vaso-occlusive pain crisis (VOC) in comparison with steady-state SCD patients and healthy controls. Platelets were immunophenotyped using different activation CD markers in a group of 34 patients with SCD. Among those SCD patients, 19 patients were admitted to our hospital for symptoms of VOC pain crises. Fifteen patients of SCD were studied in parallel during asymptomatic steady state (SS) of their disease. Fifteen healthy control volunteers were immunophenotyped in parallel. In addition, all patients and healthy contr...
Source: Journal of Hematopathology - June 1, 2018 Category: Pathology Source Type: research

Leukemic mantle cell lymphoma and chronic lymphocytic leukemia: a rare composite lymphoma and literature review
We report a case of a 75-year-old male with a slowly progressing lymphocytosis, but who was otherwise asymptomatic. Flow cytometry revealed two different populations, one showing bright Lambda positivity and CD5(+), while the other was positive for both lambda and kappa and was CD5(+). Subsequently, cytogenetics revealed two different populations, one with a trisomy 12 and the other with a t(11;14). Additionally, immunohistochemistry on a bone marrow biopsy showed SOX-11 to be negative and cyclin D1 to be positive on scattered lymphocytes. This was consistent with a composite lymphoma of leukemic mantle cell lymphoma and c...
Source: Journal of Hematopathology - June 1, 2018 Category: Pathology Source Type: research

Myeloid sarcoma of the nasal septum following invasive fungal sinusitis: report of a case and review of the relevant literature
This report describes a 75-year-old male with a known history of AML who developed both AIFS and myeloid sarcoma. Several months after successful treatment for biopsy proven AIFS, this patient developed a new soft tissue mass of the nasal septum. A new biopsy was obtained which revealed infiltrating blast cells with evidence of monocytic differentiation diagnostic of myeloid sarcoma. Given symptoms of severe nasal obstruction, this patient received palliative radiotherapy to the nasal septum, which provided temporary improvement prior to the patient succumbing to his underlying malignancy. We review the existing reports of...
Source: Journal of Hematopathology - June 1, 2018 Category: Pathology Source Type: research