| Session: Extramedullary manifestation of neoplastic myeloid disorder |
| Case number: 017 |
Submitter(s): Cherie H. Dunphy.
Clinical history
A 42 year old male, with a history of alcohol abuse and a 7-month history of myelodysplastic syndrome (MDS) associated with multiple cytogenetic abnormalities, presents with new onset retroperitoneal and cervical lymphadenopathy. He has received anti-thymocyte globulin, prednisone, and cyclosporine, as well as subsequent Thallidomide in the past for his MDS.
Current CBC and differential results:
WBC count is 400/ul with an absolute neutrophil count of 0 and an absolute lymphocyte count of 300/ul. There are 21% large unstained cells. The hemoglobin is 9.4 g/dl with an MCV of 78 FL and the platelet count is 11,000/ul.
Details of gross/microscopic pathology:
The Wright's stained touch preparation of the fresh left supraclaviuclar lymph node (Fig. 1) reveals sheets of large cells with somewhat condensed nuclear chromatin associated with dark blue cytoplasm and occasional cytoplasmic vacuoles.
The left supraclavicular lymph node is fixed in formalin. H&E sections (Fig. 2) reveal complete nodal effacement by large malignant cells with an increased mitotic rate and individual cell necrosis, as well as massive areas of necrosis.
Immunophenotype (flow cytometry/immunohistochemistry):
Flow cytometric analysis reveals 70% of cells within the gated region. Within the gated region, 74% of cells express Glycophorin A (Fig. 3, upper left and upper right quadrants) and dim CD45. No additional markers, including T-cell, B-cell, or myelomonocytic markers are expressed.
PAS stain of the touch preparation reveals distinct positivity within the cytoplasm of the malignant cells (Fig. 4, lower left region).
Hemoglobin A1 immunohistochemistry (Fig. 5) reveals unequivocal intense staining of the large malignant cells.
Immunohistochemical staining with CD45, CD20, CD3, HMB.45, S-100, AE1/AE3, S-100, kappa, lambda, and CD30 are all negative in the malignant cells.
Cytogenetics:
Cytogenetic studies reveal the abnormalities previosuly seen, including extra copies of chromosomes 5 and 8 and monosomies for chromosomes Y and 15. These cells contain an unbalanced 5;15 translocation, a deleted chromosome 8q, a deleted chromosome 10q, derivative chromomsomes 19 and 20, and isochromosome 20q. One of the 7 cells appears to be a broken tetraploid, including the above abnormalities. In addition to these changes, the karyotype now includes ring and marker chromosomes, not previously documented.
Molecular analysis:
None.
Interesting feature(s) of submitted case:
Presentation of erythroblastic sarcoma in lymph node in patient with previous history of MDS.
Proposed diagnosis:
Erythroblastic sarcoma.
Panel diagnosis:
agree with proposed diagnosis
Comments:
Stains performed by the panel: NPM- (+in nuclei), Glycophorin C+, hemoglobin-, MPO not evaluable, CD117-, lysozyme-. Additional comment from the submitter: there was no BM exam concurrent to myeloid sarcoma.
PowerPoint:
Presentation Link
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