| Session: Mast cell disease |
| Case number: 087 |
Submitter(s): Isinsu Kuzu, Cevriye Cansiz, Pervin Topcuoglu.
Clinical history
34 years old male. In January 1995 when he was 22 years old, he was diagnosed as nodular sclerosis Hodgkin's lymphoma without any bone marrow involvement. Eight courses of ABVD was performed for therapy. He was in remission untill 2004. In November 2004 he admitted to hospital with pancytopenia. Bone marrow aspiration smear revealed secondary AML. The background was containing multilineage displasia. Induction chemotherapy with cytarabine 7 days and idarubucine 3 days was performed fallowed by consolidation with 3 times high dose cytarabine. He was in remission after the therapy. He was treated by antifungal agents during chemotherapy for fungal pneumonia. Allogeneic bone marrow transplantation was planned. Three control bone marrow biopsies revealed remission for AML. Multiple nodules with maximum size of 11 mm in diameter were observed in the liver by ultrasonography. These nodules were interpreted as scars of fungal infection. Diffuse mild splenomegaly was reported on ultrasonography. He has no symptoms. Search for unrelated donor is going on.
Details of gross/microscopic pathology:
Case registration # 2006
Control bone marrow trephine biopsy 22 months after the AML therapy. The cellularity is 50-60%. There are multiple paratrabecular and intertrabecular agregates composed of histiyocytes, lymphocytes, eosinophil leukocytes without any atypical cellular elements. The aspirate smears contain 39% erytroid, 21% myelocytes and metamyelocytes, 16% stab and granulocytes, 17% lymphocytes, 1% eosinophils, 6% monocytes.
Immunophenotype (flow cytometry/immunohistochemistry):
Bone marrow trephine biopsy immunohistochemistry: The lymphocytes in the agregates were predominantly composed of CD20 positive B cells, CD3 positive T cells were scattered or cumulated around the agregates. CD68, CD117 and mast cell tryptase were strongly positive on histiocytic cells within these agregates.
Cytogenetics:
Cytogenetics at diagnosis of AML revealed t(8;21). The translocation was negative on follow up examinations and confirmed by using either conventional cytogenetics or quantitative RT-PCR.
Molecular analysis:
For Kit mutations both on AML specimen and the bone marrow at remission are pending.
Interesting feature(s) of submitted case:
Differential diagnosis of the mast cell diasease entities SM-AHNMD and tertiary ISM.
Proposed diagnosis:
Bone marrow involvement by mast cell disease.
Panel diagnosis:
Systemic mastocytosis
Comments:
Studies performed by the panel: positive for D816V mutation, CD25+
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