SH/EAHP 2007 Workshop - Progress in T-cell and NK cell Malignancies - title graphic

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Session: Mast cell disease
Case number: 048

Submitter(s): Gerald M. Penn.

Clinical history
A young female (DOB: 11-5-82) presented with a "mole" on the mid-back which felt hot and it had a history of swelling and itching after scratching. There was no hx of SOB, diarrhea, nor hives. Physical Exam was normal except for the mole, and there was no hepatosplenomegaly. A CBC showed WBC 6.0 K/uL, HGB 13.9 g/dL, MCV 91.8 fL, and PLT 313 K/uL. A differential of nucleated cells demonstrated 55.6% granulocytes, 33.2% lymphocytes, 6.5% monocytes, 4.2% eosinophils, and 0.5% basophils. A serum tryptase was 3.76 ng/mL (nl: < 11.5 ng/mL). A microscopic review of the peripheral blood smear showed a normochromic, normocytic RBC pattern with adequate platelets and no atypical cells seen.

Details of gross/microscopic pathology:
A photograph of the mid-back lesion is seen in[figure1]followed by the microscopic images.[figure2]Dense aggregates of cuboidal cells are seen in the mid and lower dermis.

Immunophenotype (flow cytometry/immunohistochemistry):
Immunohistochemistry shows that the cuboidal cells are lysozyme+, myeloperoxidase-, CD68+, and CD43+.[figure3]The cells are identified as aberrant mast cells by demonstrating that they are: CD2+, CD3-, CD117+, CD34-, and CD25+.[figure4][figure5]

Cytogenetics:
Test was not performed.

Molecular analysis:
Test was not performed.

Interesting feature(s) of submitted case:
Although the mast cells have an aberrant phenotype, the normal CBC and serum tryptase level indicates that this a SOLITARY MASTOCYTOMA and further studies are not required. This case is not for presentation, but it should be used for comparison to the aggressive systemic processes. The patient will be followed annually by total body skin examination, CBC, and serum tryptase.

Proposed diagnosis:
Solitary Mastocytoma.

Panel diagnosis:
agree with proposed diagnosis

Comments:
Panel comment: cutaneous mastocytosis/mastocytoma. Studies performed by the panel: WT c-kit, CD25+ 

Images:
Case Image 10a.jpg Figure 1
Case Image 10b.jpg Figure 2
Case Image 10c.jpg Figure 3
Case Image 10d.jpg Figure 4
Case Image 10e.jpg Figure 5

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