Hematol. Rep., Volume 4, Issue 1 (January 2012) – 5 articles

Hepatosplenic γδ T-cell lymphoma (HSTCL) is a very rare peripheral T-cell lymphoma characterized by extranodal infiltration of mature malignant post-thymic T-lymphocytes into sinusoids of the liver and spleen without lymphadenopathy and significant cytopenias. The aetiology of the disease is unknown. We describe the case of a female patient in whom HSTCL developed after delivery and who was previously without disease. Flow cytometry and liver puncture are essential for diagnosing HSTCL, especially in patients with unexplained pancytopenia and hepatosplenomegaly. Since phenotypic results can easily be misinterpreted as non-malignant, the examiner should have enough experience to recognize clonal changes of T-lymphocytes. Namely, in contrast to B-lymphocytes, T-lymphocytes do not have an efficient indicator of clonality and are recognized by flow cytometry based only on aberrant expression of commonly present antigens of T-cell and NK-cell subsets. At present, there is no known cure for HSTCL with a maximum survival up to 2 years. Full article

An 11-year-old American boy was staying with his family in Indonesia. He presented with a 5-month history of recurrent bruises and ecchymosis. A clinical diagnosis of acquired platelet dysfunction with eosinophilia was made when his full blood counts showed hypereosinophilia (7.4 × 10⁹/L) with normal platelet count and gray platelets under the microscope. The diagnosis was supported by abnormal platelet aggregation tests consistent with a storage pool disorder. The bleeding symptoms and eosinophilia resolved a month later with a full course of antihelminthic therapy. Hematologists should be aware of this unusual disease in travelers returning from the Southeast Asia. Full article

Morphological differentiation between benign and malignant lymphoproliferative disorders (LPDs) can be challenging. Immunophenotyping (IPT) by either technique, flow cytometry or immunohistochemistry (IHC), is an important step in solving such difficulty. Thirty-five newly diagnosed patients with chronic B-cell neoplasms (11 chronic lymphocytic leukemia, 22 non Hodgkin lymphoma and 2 hairy cell leukemia) were included in this study with age range from 20 to 70 years. Monoclonal antibodies surface expression using lymphoproliferative disorders panel (CD45, CD19, CD5, CD10, CD11c, CD20, CD22, CD23, CD38, CD79b, FMC7, CD103, CD25, kappa and lambda light chains) by flow cytometry was done on bone marrow samples. CD20, CD5, CD23, Bcl-2, Bcl-6, kappa and lambda light chain immunostaining were performed on fixed bone marrow trephine biopsy specimen. The sensitivity of IHC was 81.8% in chronic lymphocytic leukemia (CLL) and 100% in non Hodgkin lymphoma (NHL) as regards CD20, 100% in both groups as regards CD5, 46% in CLL and 66.7% in NHL as regards CD23, 33.3% in CLL and 50% in NHL as regards kappa chain, 20% in CLL and 33.3% in NHL as regards lambda chain. We found that IHC and flow cytometry are equally effective in diagnosing CLL; however, IHC might be slightly more sensitive than flow cytometry in detecting bone marrow infiltration in NHL and hairy cell leukemia (HCL). Full article

Polycythaemia has been reported rarely as a familial condition. There is evidence to suggest transmission as a Mendelian dominant trait, but recessive inheritance has also been described. We present here a case of benign familial polycythaemia in a 25-year-old male with similar presentation in his family members. Our patient presented with reddish discolouration of eyes, early satiety , weight loss and itching at intervals for four years. An additional examination revealed red beefy tongue and Grade III clubbing. The importance of presenting this case lies in the fact that the prognosis appears to be good in these patients, but regular observation is necessary as Kiladjian and colleagues have mentioned that there is a risk of leukaemia, thrombosis and myelofibrosis in these patients later on, as the idiopathic erythrocytosis group contains a certain number of polycythaemia patients. Full article

Mantle cell lymphoma represents 2.5–7% all of non Hodgkin’s lymphomas. Stomach is the most common site of extranodal lymphoma. However, that is not the case with mantle cell lymphoma, which is extremely rare. We present a case of 71-year-old woman admitted to the Internal Clinic of the University Clinical Hospital Center Rijeka, because of stomach discomfort and melena. Endoscopy and computed tomography revealed a polyp in gastric antrum. Histopathologic, immunohistochemic and genetic methods were also performed and the results were consistent with primary gastric mantle cell lymphoma without periepigastric and/or local or distant abdominal lymph node involvement. Full article