| Session: Myelodysplastic syndrome |
| Case number: 085 |
Submitter(s): Gerald M. Penn.
Clinical history
This elderly female (DOB: 06/11/1923) presented to the Hematologist in 08/2003 with anemia and a monoclonal gammopathy (0.5g/dL). She had a past hx of Ca of vulva in 1998 treated with radiation. A CBC showed WBC 5.9K/uL, HGB 11.3 g/dL, MCV 95.6 fL, and PLT 287K/uL. A differential of nucleated cells showed 50% granulocytes, 38% lymphocytes, 6% monocytes, and 6% eosinophils. A bone marrow performed on 08/04/2003 demonstrated a normo-cellular pattern for age with a M/E of 2:1, slight increase of megakaryocytes and frequent uninuclear forms. There were 4% plasma cells and 3% blasts. She was treated with Procrit and vitamin B12. In 09/2005, the patient returned due to anemia. The CBC showed WBC 6.1K/uL, HGB 9.9 g/dL, MCV 100 fL, and PLT 589K/uL. A differential of nucleated cells showed 62% granulocytes, 25% lymphocytes, 7% monocytes, 4% eosinophils, and 2% basophils. A bone marrow performed on 09/28/2005 demonstrated a normo-cellular pattern with increased megakaryocytes, many having a uninuclear morphology, M/E of 3.5:1, 4.5% plasma cells, and 3% blasts.
Details of gross/microscopic pathology:
The bone marrow aspirate of 08/04/2003 demonstrated uninuclear megakaryocytes,[figure1]monomorphic plasma cells,[figure2]and increased myeloblasts.[figure3]Similar results were also seen in 09/2005. The bone marrow biopsy of 2003 (fixed in neutral buffered formalin and decalcified with acid/edta), showed a normo-cellular pattern for age, uninuclear megakaryocytosis, and increased plasma cells (CD 138 stain) without nodules or significant clustering.[figure4]
Immunophenotype (flow cytometry/immunohistochemistry):
The bone marrow biopsy of 2005 demonstrated a similar cellularity, erythroid hypoplasia (glycophorin A stain), lack of significant reticulin, and plasmacytosis (BCL-2 stain).[figure5]Flow Cytometric analysis of the 2005 aspirate demonstrated decreased side-scatter of the myeloid cells, increased myeloid precursors, and confirmed the plasmacytosis.[figure6]The myeloid precursors generate a tight-cluster with CD 34 and HLA-DR. A subpopulation of the myeloblasts and myeloid cells (red arrow) as well as the plasma cells (black arrow) stain with CD 56.[figure7]A comparison of the 2003 and 2005 cellularity, plasmacytosis (CD 56 stain), and number of myeloblasts (CD 34 stain) is seen in Figure 8.[figure8]
Cytogenetics:
Both the 2003 and 2005 bone marrow aspirates demonstated:
46,XX,del(5)(q13q33)[19]/46,XX[1].
Molecular analysis:
A FISH panel for myelodysplasia showed only 5q-.[figure9]
Interesting feature(s) of submitted case:
Although 5q- syndrome may cause anemia, there is insufficient progression of the myelodysplasia to account for the additional decrease in HGB. A search for other etiological factors demonstrated a perirectal mass with liver metastases (vulvar primary). Patient died in Hospice with no evidence of high grade MDS or myeloma.
Proposed diagnosis:
Monoclonal gammopathy of unknown significance with 5q- syndrome and anemia of chronic disease.
Panel diagnosis:
agree with proposed diagnosis
Comments:
Stains performed by the panel: CD34 within normal limits.
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