blood Sickle Cell Disease Articles

Forced chromatin looping raises fetal hemoglobin in adult sickle cells to higher levels than pharmacologic inducers
Laura Breda, Irene Motta, Silvia Lourenco, Chiara Gemmo, Wulan Deng, Jeremy W. Rupon, Osheiza Y. Abdulmalik, Deepa Manwani, Gerd A. Blobel, Stefano Rivella
Aug 25, 2016
Customizing the genome as therapy for the β-hemoglobinopathies
Matthew C. Canver, Stuart H. Orkin
May 26, 2016
Elevated D-dimer levels in African Americans with sickle cell trait
Rakhi P. Naik, James G. Wilson, Lynette Ekunwe, Stanford Mwasongwe, Qing Duan, Yun Li, Adolfo Correa, Alexander P. Reiner
May 05, 2016
Silent cerebral infarcts and cerebral aneurysms are prevalent in adults with sickle cell anemia
Adetola A. Kassim, Sumit Pruthi, Matthew Day, Mark Rodeghier, Melissa C. Gindville, Max A. Brodsky, Michael R. DeBaun, Lori C. Jordan
Apr 21, 2016
Long-term treatment follow-up of children with sickle cell disease monitored with abnormal transcranial Doppler velocities
Françoise Bernaudin, Suzanne Verlhac, Cécile Arnaud, Annie Kamdem, Isabelle Hau, Emmanuella Leveillé, Manuela Vasile, Florence Kasbi, Fouad Madhi, Christine Fourmaux, Sandra Biscardi, Eliane Gluckman, Gérard Socié, Jean-Hugues Dalle, Ralph Epaud, Corinne Pondarré
Apr 07, 2016
Thrombin-independent contribution of tissue factor to inflammation and cardiac hypertrophy in a mouse model of sickle cell disease
Erica M. Sparkenbaugh, Pichika Chantrathammachart, Kasemsiri Chandarajoti, Nigel Mackman, Nigel S. Key, Rafal Pawlinski
Mar 10, 2016
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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

Forced chromatin looping raises fetal hemoglobin in adult sickle cells to higher levels than pharmacologic inducers
Laura Breda, Irene Motta, Silvia Lourenco, Chiara Gemmo, Wulan Deng, Jeremy W. Rupon, Osheiza Y. Abdulmalik, Deepa Manwani, Gerd A. Blobel, Stefano Rivella
Aug 25, 2016
Customizing the genome as therapy for the β-hemoglobinopathies
Matthew C. Canver, Stuart H. Orkin
May 26, 2016
View More Articles »

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