From bloodjournal.hematologylibrary.org by guest on June 27, 2014. For personal use only.
2014 123: 2449 doi:10.1182/blood-2014-01-550657
Hemophagocytic lymphohistiocytosis and disseminated histoplasmosis Alina M. Huang
Updated information and services can be found at: http://bloodjournal.hematologylibrary.org/content/123/16/2449.full.html Articles on similar topics can be found in the following Blood collections BloodWork (303 articles) Free Research Articles (2492 articles) Phagocytes, Granulocytes, and Myelopoiesis (462 articles) Information about reproducing this article in parts or in its entirety may be found online at: http://bloodjournal.hematologylibrary.org/site/misc/rights.xhtml#repub_requests Information about ordering reprints may be found online at: http://bloodjournal.hematologylibrary.org/site/misc/rights.xhtml#reprints Information about subscriptions and ASH membership may be found online at: http://bloodjournal.hematologylibrary.org/site/subscriptions/index.xhtml
Blood (print ISSN 0006-4971, online ISSN 1528-0020), is published weekly by the American Society of Hematology, 2021 L St, NW, Suite 900, Washington DC 20036. Copyright 2011 by The American Society of Hematology; all rights reserved.
From bloodjournal.hematologylibrary.org by guest on June 27, 2014. For personal use only.
bloodwork ®
IMAGES
IN HEMATOLOGY
Hemophagocytic lymphohistiocytosis and disseminated histoplasmosis
c Alina M. Huang, Brown University
A
25-year-old Guatemalan man presented to the hospital with abdominal distension and fever. A computed tomography scan showed marked hepatosplenomegaly and bulky abdominal adenopathy. A complete blood count showed pancytopenia: white blood count, 7.7 3 109/L; hemoglobin, 7.0 g/dL; platelets, 48 3 109/L. White blood cell inclusions were present. Further work-up revealed AIDS with a CD4 count of 4 cells/mL. Evaluation of his anemia revealed a ferritin level of 85 000 ng/mL, which was a level that prompted consideration of hemophagocytic lymphohistiocytosis (HLH) as a unifying diagnosis. He fulfilled 5 of 8 diagnostic criteria: fever $38.5°C, splenomegaly, $2 cytopenias, ferritin level .500 ng/mL, and evidence of hemophagocytosis in the bone marrow. Prominent clusters of Histoplasma capsulatum were seen disseminated throughout the marrow aspirate (panel A). Serologic testing for Histoplasma antigen was positve. Hemophagocytosis was seen (panel B), in which a histiocyte is engulfing a red blood cell and several Histoplasma organisms. HLH is a syndrome of pathologic immune activation characterized as primary or secondary in etiology. This patient had HLH secondary to disseminated histoplasmosis in the setting of severe immune deficiency. He received dexamethasone for treatment of HLH, as well as highly active antiretroviral therapy and antifungal treatment to treat the underlying infectious etiology. He recovered without incident and is doing well.
For additional images, visit the ASH IMAGE BANK, a reference and teaching tool that is continually updated with new atlas and case study images. For more information visit http://imagebank.hematology.org. © 2014 by The American Society of Hematology BLOOD, 17 APRIL 2014 x VOLUME 123, NUMBER 16
2449
2014 123: 2449 doi:10.1182/blood-2014-01-550657
Hemophagocytic lymphohistiocytosis and disseminated histoplasmosis Alina M. Huang
Updated information and services can be found at: http://bloodjournal.hematologylibrary.org/content/123/16/2449.full.html Articles on similar topics can be found in the following Blood collections BloodWork (303 articles) Free Research Articles (2492 articles) Phagocytes, Granulocytes, and Myelopoiesis (462 articles) Information about reproducing this article in parts or in its entirety may be found online at: http://bloodjournal.hematologylibrary.org/site/misc/rights.xhtml#repub_requests Information about ordering reprints may be found online at: http://bloodjournal.hematologylibrary.org/site/misc/rights.xhtml#reprints Information about subscriptions and ASH membership may be found online at: http://bloodjournal.hematologylibrary.org/site/subscriptions/index.xhtml
Blood (print ISSN 0006-4971, online ISSN 1528-0020), is published weekly by the American Society of Hematology, 2021 L St, NW, Suite 900, Washington DC 20036. Copyright 2011 by The American Society of Hematology; all rights reserved.
From bloodjournal.hematologylibrary.org by guest on June 27, 2014. For personal use only.
bloodwork ®
IMAGES
IN HEMATOLOGY
Hemophagocytic lymphohistiocytosis and disseminated histoplasmosis
c Alina M. Huang, Brown University
A
25-year-old Guatemalan man presented to the hospital with abdominal distension and fever. A computed tomography scan showed marked hepatosplenomegaly and bulky abdominal adenopathy. A complete blood count showed pancytopenia: white blood count, 7.7 3 109/L; hemoglobin, 7.0 g/dL; platelets, 48 3 109/L. White blood cell inclusions were present. Further work-up revealed AIDS with a CD4 count of 4 cells/mL. Evaluation of his anemia revealed a ferritin level of 85 000 ng/mL, which was a level that prompted consideration of hemophagocytic lymphohistiocytosis (HLH) as a unifying diagnosis. He fulfilled 5 of 8 diagnostic criteria: fever $38.5°C, splenomegaly, $2 cytopenias, ferritin level .500 ng/mL, and evidence of hemophagocytosis in the bone marrow. Prominent clusters of Histoplasma capsulatum were seen disseminated throughout the marrow aspirate (panel A). Serologic testing for Histoplasma antigen was positve. Hemophagocytosis was seen (panel B), in which a histiocyte is engulfing a red blood cell and several Histoplasma organisms. HLH is a syndrome of pathologic immune activation characterized as primary or secondary in etiology. This patient had HLH secondary to disseminated histoplasmosis in the setting of severe immune deficiency. He received dexamethasone for treatment of HLH, as well as highly active antiretroviral therapy and antifungal treatment to treat the underlying infectious etiology. He recovered without incident and is doing well.
For additional images, visit the ASH IMAGE BANK, a reference and teaching tool that is continually updated with new atlas and case study images. For more information visit http://imagebank.hematology.org. © 2014 by The American Society of Hematology BLOOD, 17 APRIL 2014 x VOLUME 123, NUMBER 16
2449
