| Session: Mast cell disease |
| Case number: 152 |
Submitter(s): Stefano A. Pileri, Elena Sabattini, Cristina Campidelli, Claudio Agostinelli, Francesco Bacci.
Clinical history
A 62-year-old woman with an unremarkable personal history was found to have an unsymptomatic IgG/kappa monoclonal gammpathy in 1999 in the absence of scheletric lesions or systemic involvement. She was followed at another hospital until 2004, when she moved to our institution where she has been periodically checked ever since. Laboratory investigation have confirmed the IgG increase (last control value: 5,700) with good peripeheral blood values (WBC 3.940/mm3, Hg 10.9 g/dl, platelets 166.000/mm3) with persistent negativity for pathological bone or organ involvement at MNR, CAT, and PET. A bone-marrow biopsy was performed in 2005 (sample submitted). The patient is still off-therapy and in good health conditions.
Details of gross/microscopic pathology:
Hypocellular marrow (15% cellularity) with plasma cell infiltrate provided with mature cytology (Marschalko type) that grew both in small aggregates and in single interstitial cells. This proliferation occupied about 20% of marrow cellularity and could be staged as 1A according to Bartl and Frisch 1982.
Several foci of spindle shaped cells with clear cytoplasm and oval slightly indented nucleus could be observed, at times in close contact with small lymphoid nodules, eosinophils, and plasma cells.
The residual marrow showed a regular haemopoietic composition.
The bone marrow biopsy had been fixed in B5 and decalcified in an EDTA-based solution.
Immunophenotype (flow cytometry/immunohistochemistry):
Plasma cells: monotypic restriction for kappa light chain.
Lymphocytes: abundant T cells (CD3 positive) and aggregates of B-lymphocytes (CD20 positive).
Spindle cells: positive for triptase and cKIT/CD117.
Cytogenetics:
Not performed.
Molecular analysis:
Not performed.
Interesting feature(s) of submitted case:
1) Rare association between plasma cell dyscrasia and mastocytosis.
2) Although morphologically neoplastic, the mastocellular infiltrate does not affect clinics.
Proposed diagnosis:
Association between plasma cell myeloma (stage 1A) and mastocytosis.
Panel diagnosis:
SM-AHNMD/plasma cell myeloma
Comments:
Studies performed by the panel: WT c-kit, CD25+
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