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by Karthik A. Ganapathi, Danielle M. Townsley, Amy P. Hsu, Diane C

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Presentation on theme: "by Karthik A. Ganapathi, Danielle M. Townsley, Amy P. Hsu, Diane C"— Presentation transcript:

1 GATA2 deficiency-associated bone marrow disorder differs from idiopathic aplastic anemia
by Karthik A. Ganapathi, Danielle M. Townsley, Amy P. Hsu, Diane C. Arthur, Christa S. Zerbe, Jennifer Cuellar-Rodriguez, Dennis D. Hickstein, Sergio D. Rosenzweig, Raul C. Braylan, Neal S. Young, Steven M. Holland, and Katherine R. Calvo Blood Volume 125(1):56-70 January 1, 2015 ©2015 by American Society of Hematology

2 Peripheral blood indices of GATA2 and AA patients.
Peripheral blood indices of GATA2 and AA patients. (A) GATA2 and AA patients have comparable ages, hemoglobin levels, and WBC counts, but GATA2 patients have higher platelet, neutrophil count, and lower lymphocyte count compared with AA patients. (B) GATA2 patients have reduced peripheral blood monocytes, B cells, and NK cells, and relatively increased T cells compared with AA patients. Karthik A. Ganapathi et al. Blood 2015;125:56-70 ©2015 by American Society of Hematology

3 Bone marrow flow cytometric analysis of monocytes and lymphoid subsets in GATA2 and AA patients.
Bone marrow flow cytometric analysis of monocytes and lymphoid subsets in GATA2 and AA patients. (A) GATA2 patients have reduced bone marrow monocytes (CD33+, HLA-DR+), mature B cells (CD19+, CD20+), and NK cells (sCD3−, CD56+) compared with AA patients. (B) A subset of GATA2 patients has atypical CD19−CD56+ plasma cells (gating performed on CD38+, CD138+ positive population), which are not identified in AA patients. Karthik A. Ganapathi et al. Blood 2015;125:56-70 ©2015 by American Society of Hematology

4 Comparison of T-cell subsets between GATA2 and AA patients.
Comparison of T-cell subsets between GATA2 and AA patients. (A) GATA2 patients have relatively increased bone marrow T cells, (B) inverted CD4:CD8 ratios, (C) increased CD3+CD56+, and (D) increased CD3+CD57+ T-cell subsets compared with AA patients. Karthik A. Ganapathi et al. Blood 2015;125:56-70 ©2015 by American Society of Hematology

5 Comparison of bone marrow precursor cells between GATA2 and AA patients.
Comparison of bone marrow precursor cells between GATA2 and AA patients. (A) GATA2 and AA patients have comparable levels of CD34-positive cells. (B) GATA2 patients have absent hematogones compared with AA patients. Karthik A. Ganapathi et al. Blood 2015;125:56-70 ©2015 by American Society of Hematology

6 Comparison of peripheral blood and bone marrow morphologic and immunohistochemical features between GATA2 and AA patients. Comparison of peripheral blood and bone marrow morphologic and immunohistochemical features between GATA2 and AA patients. GATA2 patient marrows have greater overall cellularity, increased megakaryocytes with atypical features (highlighted by CD61), but overall similar numbers of CD34-positive cells compared with AA marrows. A subset of GATA2 patients has hypogranular circulating neutrophils. Karthik A. Ganapathi et al. Blood 2015;125:56-70 ©2015 by American Society of Hematology


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