blood Sickle Cell Disease Articles

Reduced PU.1 expression underlies aberrant neutrophil maturation and function in β-thalassemia mice and patients
Panjaree Siwaponanan, Jurre Ynze Siegers, Razi Ghazali, Thian Ng, Bradley McColl, Garrett Zhen-Wei Ng, Philip Sutton, Nancy Wang, Isabelle Ooi, Chayada Thiengtavor, Suthat Fucharoen, Pornthip Chaichompoo, Saovaros Svasti, Odilia Wijburg, Jim Vadolas
Jun 08, 2017
Association of circulating transcriptomic profiles with mortality in sickle cell disease
Ankit A. Desai, Zhengdeng Lei, Neil Bahroos, Mark Maienschein-Cline, Santosh L. Saraf, Xu Zhang, Binal N. Shah, Seyed M. Nouraie, Taimur Abbasi, Amit R. Patel, Roberto M. Lang, Yves Lussier, Joe G. N. Garcia, Victor R. Gordeuk, Roberto F. Machado
Jun 01, 2017
Treating sickle cell disease by targeting HbS polymerization
William A. Eaton, H. Franklin Bunn
May 18, 2017
Sickle cell disease: an international survey of results of HLA-identical sibling hematopoietic stem cell transplantation
Eliane Gluckman, Barbara Cappelli, Francoise Bernaudin, Myriam Labopin, Fernanda Volt, Jeanette Carreras, Belinda Pinto Simões, Alina Ferster, Sophie Dupont, Josu de la Fuente, Jean-Hugues Dalle, Marco Zecca, Mark C. Walters, Lakshmanan Krishnamurti, Monica Bhatia, Kathryn Leung, Gregory Yanik, Joanne Kurtzberg, Nathalie Dhedin, Mathieu Kuentz, Gerard Michel, Jane Apperley, Patrick Lutz, Bénédicte Neven, Yves Bertrand, Jean Pierre Vannier, Mouhab Ayas, Marina Cavazzana, Susanne Matthes-Martin, Vanderson Rocha, Hanadi Elayoubi, Chantal Kenzey, Peter Bader, Franco Locatelli, Annalisa Ruggeri, Mary Eapen
Mar 16, 2017
Association among sickle cell trait, fitness, and cardiovascular risk factors in CARDIA
Robert I. Liem, Cheeling Chan, Thanh-Huyen T. Vu, Myriam Fornage, Alexis A. Thompson, Kiang Liu, Mercedes R. Carnethon
Feb 09, 2017
Sickle cell trait is not associated with an increased risk of heart failure or abnormalities of cardiac structure and function
Natalie A. Bello, Hyacinth I. Hyacinth, Nicholas S. Roetker, Samantha R. Seals, Rakhi P. Naik, Vimal K. Derebail, Abhijit V. Kshirsagar, Nigel S. Key, James G. Wilson, Adolfo Correa, Robert J. Adams, Leonard D. Egede, W. T. Longstreth Jr, Gaurav Choudhary, Beatrice E. Gee, Austin L. Hughes, Amil M. Shah, JoAnn E. Manson, Matthew Allison, Gregory L. Burke, Aaron R. Folsom, Cara L. Carty, Alexander P. Reiner, Scott D. Solomon, Suma H. Konety
Feb 09, 2017
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ASH ® Image Bank

Pappenheimer Bodies in Sickle Cell Disease

 

A 26-year-old AF male with sickle cell disease who presented to the ER complaining of abdominal pain. His CBC at that time showed a hemoglobin of 7.2g/dL, WBC of 11.2k/uL and a platelet count of 360k/uL. His peripheral smear is show. Sickle cells are readily identified (intermediate-sized arrow) throughout the smear. A RBC with a Howell-Jolly body (small arrow) is also noted. Multiple irregular inclusions (large arrow) are present in many of the other RBC. These iron-containing granules are Pappenheimer bodies. A Prussian Blue is needed to confirm the presence of non-heme iron in these granules. Also note on this smear increased polychromatophilic cells and target cells.

Fish mouth vertebrae and sickle cell spine

 

A 22-year-old male with sickle cell disease (SCD) presented to the emergency department with a chief complaint of acute left lower extremity pain and a non-productive cough. PA and lateral chest roentograms were obtained revealing biconcave vertebral defects or "fish mouth" vertebrae. Developing in greater than seventy percent of patients with SCD, the depicted vertebral changes result from infarction of the vertebral bodies and thinned trabeculae allowing hyperplastic marrow to expand into the central vertebral space (solid arrows, Panels A and B). Debate exists regarding appropriate terminology as "fish" or "fish mouth" vertebrae. Though the appearance of the intervertebral disks resembles a fish mouth, the biconcave vertebral bodies are strikingly similar to a salmon’s vertebrae (dashed arrow, Panel C*).

Aplastic Crisis in a Patient with Sickle Cell Disease

 

Numerous sickled RBC's are present (small arrows). A single nucleated RBC is noted (large arrow). Of note is the absence of polychromasia.

Sickle cell disease – RBC morphology

 

Peripheral smear from a patient with sickle cell disease illustrates the spectrum of RBC findings in this disorder including sickle cells, polychromatophilic RBCs, target cells, and Howell-Jolly bodies.

Sickle cell disease – RBC morphology - 2

 

In addition to the RBC findings noted in Fig. 1, this image shows a nucleated RBC (arrow).

Sickle cell disease – RBC morphology - 4

 

Crescent-shaped sickle cells are noted (double arrow). Several RBCs with Howell-Jolly bodies are also present. One has two such inclusions (single arrow).

Pappenheimer bodies and basophilic stippling in sickle cell disease

 

The Pappenheimer bodies are shown at high magnification. The inclusion in the RBC at the top center of the image has the appearance of a Howell-Jolly body.

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blood How I Treat

How I treat recurrent venous thromboembolism in patients receiving anticoagulant therapy
Sam Schulman
Jun 22, 2017
Reduced PU.1 expression underlies aberrant neutrophil maturation and function in β-thalassemia mice and patients
Panjaree Siwaponanan, Jurre Ynze Siegers, Razi Ghazali, Thian Ng, Bradley McColl, Garrett Zhen-Wei Ng, Philip Sutton, Nancy Wang, Isabelle Ooi, Chayada Thiengtavor, Suthat Fucharoen, Pornthip Chaichompoo, Saovaros Svasti, Odilia Wijburg, Jim Vadolas
Jun 08, 2017
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My Say

Sickle cell disease has come full circle

Swee Lay Thein, MB, BS, FRCP, FRCPath, DSc, FMedSci
Professor of Molecular Haematology / Consultant Haematologist
King's College London School of Medicine / King's College Hospital
London, UK

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Consult a Colleague

Consult a Colleague is a service for ASH® members that helps facilitate the exchange of information between hematologists and their peers.

 

 

Feature Editor: Dr. Swee Lay Thein