blood Sickle Cell Disease Articles

How I safely transfuse patients with sickle-cell disease and manage delayed hemolytic transfusion reactions
France Pirenne, Karina Yazdanbakhsh
Jun 21, 2018
Induction of fetal hemoglobin synthesis by CRISPR/Cas9-mediated editing of the human β-globin locus
Chiara Antoniani, Vasco Meneghini, Annalisa Lattanzi, Tristan Felix, Oriana Romano, Elisa Magrin, Leslie Weber, Giulia Pavani, Sara El Hoss, Ryo Kurita, Yukio Nakamura, Thomas J. Cradick, Ante S. Lundberg, Matthew Porteus, Mario Amendola, Wassim El Nemer, Marina Cavazzana, Fulvio Mavilio, Annarita Miccio
Mar 08, 2018
Loss of NRF2 function exacerbates the pathophysiology of sickle cell disease in a transgenic mouse model
Xingguo Zhu, Caixia Xi, Bobby Thomas, Betty S. Pace
Feb 01, 2018
Red cell exchange transfusions lower cerebral blood flow and oxygen extraction fraction in pediatric sickle cell anemia
Kristin P. Guilliams, Melanie E. Fields, Dustin K. Ragan, Cihat Eldeniz, Michael M. Binkley, Yasheng Chen, Liam S. Comiskey, Allan Doctor, Monica L. Hulbert, Joshua S. Shimony, Katie D. Vo, Robert C. McKinstry, Hongyu An, Jin-Moo Lee, Andria L. Ford
Dec 18, 2017
Extracellular fluid tonicity impacts sickle red blood cell deformability and adhesion
Marcus A. Carden, Meredith E. Fay, Xinran Lu, Robert G. Mannino, Yumiko Sakurai, Jordan C. Ciciliano, Caroline E. Hansen, Satheesh Chonat, Clinton H. Joiner, David K. Wood, Wilbur A. Lam
Dec 14, 2017
Novel use Of Hydroxyurea in an African Region with Malaria (NOHARM): a trial for children with sickle cell anemia
Robert O. Opoka, Christopher M. Ndugwa, Teresa S. Latham, Adam Lane, Heather A. Hume, Phillip Kasirye, James S. Hodges, Russell E. Ware, Chandy C. John
Dec 14, 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 myelofibrosis after failure of JAK inhibitors
Animesh Pardanani, Ayalew Tefferi
Aug 02, 2018
How I treat Burkitt lymphoma in children, adolescents, and young adults in sub-Saharan Africa
Satish Gopal, Thomas G. Gross
Jul 19, 2018
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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