| Session: Extramedullary manifestation of neoplastic myeloid disorder |
| Case number: 010 |
Submitter(s): Kai Zhang.
Clinical history
38 yrs old male with leukocytosis and progressive cervical lymph node (LN) enlargement for several weeks. CBC showed: 152.5K/ul, 31% seg, 11% lymphs, 17% monos, 1% basophils, 2% metas, 2% myelos, and 10% blasts; and platelets, 248K/dl; and Hb, 13.5g/dl. BM and cervical lymph nodes bxes were obtained.No prior history of malignancy. Current medications: Zantac, Keflex, felodipine, allopurinol.
Details of gross/microscopic pathology:
Cervical LN Bx (10% formalin fixation): nodal architectural effacement with an expansion of interfollicular spaces by a population of plasmacytoid monocytes forming large aggregates.
BM aspirate: Myeloid or granulocytic hyperplasia with increased megs% and some blasts and focal areas of plasmocytoid monocytes.
Blood film: Granuocytosis with left shift and some immature cells.
Immunophenotype (flow cytometry/immunohistochemistry):
Cervical LN (abnormal plasmacytoid monocytes):
FCM: CD45+, CD4+, DR+, CD43+, CD10+, CD5+(low), CD7+ (low), CD33 (subset).
IHC: CD4+, CD68+, lysozeme+, and weakly positive for CD45, CD43, CD7, CD10.
Note: Negative for CD34, CD56, CD16, CD57, CD19, CD20, Light chains, CD3, CD1a, CD2, CD8 by FCM and IHC. Some CD34+, CD99+ and MPO+ precursors noted by IHC.
Cytogenetics:
Bone marrow aspirate: Normal, XY.
Molecular analysis:
LN: Negative for TCR (gamma) by PCR.
Interesting feature(s) of submitted case:
MPD with turmor proliferation of plasmacytoid monocytes in lymph node.
Proposed diagnosis:
Cervical LN: Classical example of uncommon case of MPD with turmor proliferation of plasmacytoid monocytes.
BM biopsy: Chronic myeloproliferative disorder (possibly CMML).
Panel diagnosis:
Plasmacytoid dendritic tumor (PDT) in MDS/MPD (CMML)
Comments:
Stains performed by the panel: HECA + (weak), CD123+, MPO-, NPM-(+in nuclei)
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