Hairy cell leukemia and increased mast cell and plasma cells in bone marrow: a case report

Background: The hairy cell leukemia (HCL) and also HCL-like disorders, including the HCL variants are a heterogeneous group of the mature lymphoid B-cell disorders that ischaracterized by the identification of hairy cells plus a specific genetic profile, also different clinical presentations, and the need for an appropriate therapy as an uncommonhematological malignancy characterized by pancytopenia. Atypical presentation of hairy cell leukemia is difficult diagnostic challenges for clinicians Case report: we report an atypical case of hairy cell leukemia with diffusing aspects. A 44-year-old man was admitted in hospital for weakness and fatigue and watery non-bloody diarrhea. The laboratory tests showed pancytopenia leading to referring for aspiration and biopsy from bone marrow. In aspiration there were medium to large cells and widespread cytoplasm and oval nuclei similar to monocyte nuclei (kidney-form) accompanied with increased number of mast-cells and plasma cells. There were no hairy projections on surface of cytoplasms and the chromatin was homogenous. In clinical examination the spleen edge was touched and the mobile 0.5*0.5 centimeter sub-mandibular gland was present. In laboratory studies it was seen that leukocyte count was 1200/mm3; hemoglobin 6.4 g/dl, platelet count 4000/mm3. Increased gamma-globulin was seen in electrophoresis and the IgA and IgG were raised. In urinalysis there was no proteinuria and Bence-Jones protein was negative. In fluocytometry there were neoplastic cells that were positive for cd19-fmc7 – coexpresion CD20/CD25 –CD11C/CD 22-CD 103 markers. In immunohistochemical staining the mast-cells were positive for CD117 and plasmacells were positive for CD138 and the hairy cells were positive for CD20. Totally, Distinct diagnosis of hairy cell leukemia variants would result in prompt treatment in these patients accompanied with better prognosis and higher therapeutic response.

کلیدواژه ها:

hairy cell leukemia ، mast cells ، plasma cells ، bone marrow