BMW3: 62 YEAR OLD AFRICAN AMERICAN FEMALE WITH LYMPHADENOPATHY OF ONE
MONTHS DURATION
Brian W Olsen1 and Wanderly De Mendoza Calaca2
1.Ingham Regional Medical Center, Dept of Pathology, Lansing, USA
2.Sparrow Regional Medical Center, Dept of Pathology, Lansing, USA
brian.olsen@irmc.org
Clinical history: 62 year old African American female presented 09/2009 with progressive, generalized
lymphadenopathy of one months duration; otherwise asymptomatic. On examination, enlarged cervical, supraclavicular,
axillary and inguinal nodes were found. She underwent bone marrow biopsy in 09/2009, and lymph node biopsy in
10/2009. Hb 10.8 grams/dL, WBC 22.1 x 109/L, Plt 35 X 109/L. Other lab. Parameters: Neutrophils 53%, Bands 6%,
Lymphocytes 19%, Monocytes 10%, Eosinophils 10%, Metamyelocytes 1%, Myelocytes 1%.
Biopsies: Bone marrow biopsy fixed in formalin, lymph node biopsy fixed in formalin.
Histopathology: Marked bone marrow cellularity, approaching 100%, with marked myeloid excess, including a relative
increase in eosinophilic precursors. Scattered, loose collections of immature cells, reminiscent of proerythroblasts.
Megakaryocytes and mature erythroid elements inconspicuous. Moderate reticulin fibrosis (2+). Lymph node with partial
effacement of tissue architecture. Prominent blastic proliferation having features reminiscent of proerythroblasts, with
some evidence of erythroid maturation. Lesser component of maturing myeloid elements, with relative prominence of
eosinophilic precursors. Benign microvascular proliferation. Residual, uninvolved lymph node including reactive
germinal centers and patent sinuses. Bone marrow: dry tap. Lymph node touch imprints: Abundance of erythroid
elements, shifted to immaturity, including an increased proportion of proerythroblasts. Maturing erythroid elements with
features of dysplasia. Atypical, maturing myelomonocytic cells, with increased proportion of eosinophilic precursors.
Rare cells suggestive of dysplastic megakaryocytes. Peripheral blood: Tear drop poikylocytosis, nucleated RBCs including cells showing nuclear irregularity - some elliptocytes ands stomatocytes, rare blast forms, scattered
promyelocytes. Both monocytosis and eosinophilia present. Rare megakaryocyte fragments, absent additional evidence
of platelet atypia.
Immunophenotype/immunohistochemistry: Lymph node: Blast forms positive for hemoglobin, glycophorin A, CD43,
CD117 (sub-set, weak), ecadherin by immunohistochemistry. Negative for markers including myeloperoxidase, CD34,
TdT, CD1a, CD3, CD20, PAX5, lysozyme, mast cell tryptase, CD68, CD61, CD30, CD11c, CD13, CD33 all by
immunohistochemistry. Vascular proliferation positive for CD34, negative for HHV8. Bone marrow: Blast forms
negative for CD34, myeloperoxidase and CD68 by immunohistochemistry.
Cytogenetics: Lymph node: 46, XX, t(5;21)(q23;p11.2), del(8)(p21)[20} by conventional cytogenetic analysis.
Peripheral blood: Negative for BCR-ABL1, PDGFRA, PDGFRB rearrangement by interphase FISH.
Molecular analysis: Peripheral blood: JAK2 V617F mutation negative by allele specific PCR.
Proposed diagnosis
Myeloid sarcoma with marked erythroblastic differentiation (lymph node), in transformation from a
myelodysplastic/myeloproliferative neoplasm consistent with CMMl with eosinophilia.
Interesting feature(s) of the submitted case
Features were judged most consistent with a rare example of an "erythroblastic sarcoma" arising in the background of
peripheral blood and bone marrow findings reminiscent of CMML with eosinophilia. Interestingly, as most recent
follow-up in 01/2010, the patient remains asymptomatic, and the lymphadenopathy noted at presentation is said to have
resolved. CBC data obtained during the most recent visit include: Hb 11.4, WBC 8.9, plt 60 X 109/L.
Other comments: Thanks to Drs. Ahmet Dogan and David Viswanatha, at the Mayo Clinic, Rochester, Minnesota, for
reviewing the lymph node biopsy material, and for performance of immunohistochemical stains, including hemoglobin
and glycophorin A, and for performance of interphase FISH studies.