blood Sickle Cell Disease Articles

How I diagnose and treat venous thromboembolism in sickle cell disease
Arun S. Shet, Ted Wun
Oct 25, 2018
How I treat the older adult with sickle cell disease
Swee Lay Thein, Jo Howard
Oct 25, 2018
How I treat hypoxia in adults with hemoglobinopathies and hemolytic disorders
Evans M. Machogu, Roberto F. Machado
Oct 25, 2018
Silent infarcts in sickle cell disease occur in the border zone region and are associated with low cerebral blood flow
Andria L. Ford, Dustin K. Ragan, Slim Fellah, Michael M. Binkley, Melanie E. Fields, Kristin P. Guilliams, Hongyu An, Lori C. Jordan, Robert C. McKinstry, Jin-Moo Lee, Michael R. DeBaun
Oct 18, 2018
Increased brain iron deposition in patients with sickle cell disease: an MRI quantitative susceptibility mapping study
Xin Miao, Soyoung Choi, Benita Tamrazi, Yaqiong Chai, Chau Vu, Thomas D. Coates, John C. Wood
Oct 11, 2018
HMOX1 and acute kidney injury in sickle cell anemia
Santosh L. Saraf, Maya Viner, Ariel Rischall, Rasha Raslan, Binal N. Shah, Xu Zhang, Jin Han, Michel Gowhari, Shivi Jain, Robert E. Molokie, Roberto F. Machado, James P. Lash, Victor R. Gordeuk
Oct 11, 2018
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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 the blast phase of Philadelphia chromosome–negative myeloproliferative neoplasms
Olatoyosi Odenike
Nov 29, 2018
How I treat breast implant–associated anaplastic large cell lymphoma
Neha Mehta-Shah, Mark W. Clemens, Steven M. Horwitz
Nov 01, 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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Feature Editor: Dr. Swee Lay Thein