blood Sickle Cell Disease Articles

Fetal hemoglobin in sickle cell anemia: a glass half full?
Martin H. Steinberg , David H. K. Chui , George J. Dover , Paola Sebastiani , Abdulrahman Alsultan
Jan 23, 2014
Heme triggers TLR4 signaling leading to endothelial cell activation and vaso-occlusion in murine sickle cell disease
John D. Belcher , Chunsheng Chen , Julia Nguyen , Liming Milbauer , Fuad Abdulla , Abdu I. Alayash , Ann Smith , Karl A. Nath , Robert P. Hebbel , Gregory M. Vercellotti
Jan 16, 2014
Vaso-occlusion in sickle cell disease: pathophysiology and novel targeted therapies
Deepa Manwani , Paul S. Frenette
Dec 05, 2013
Rare complete loss of function provides insight into a pleiotropic genome-wide association study locus
Vijay G. Sankaran , Mugdha Joshi , Akshat Agrawal , Klaus Schmitz-Abe , Meghan C. Towne , Nicholas Marinakis , Kyriacos Markianos , Gerard T. Berry , Pankaj B. Agrawal
Nov 28, 2013
HbC disorders
Martin H. Steinberg , David H. K. Chui
Nov 21, 2013
MP4CO, a pegylated hemoglobin saturated with carbon monoxide, is a modulator of HO-1, inflammation, and vaso-occlusion in transgenic sickle mice
John D. Belcher , Mark Young , Chunsheng Chen , Julia Nguyen , Kenneth Burhop , Phuc Tran , Gregory M. Vercellotti
Oct 10, 2013
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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 transfusional iron overload
A. Victor Hoffbrand , Ali Taher , Maria Domenica Cappellini
Nov 01, 2012
How I treat acute chest syndrome in children with sickle cell disease
Scott T. Miller
May 19, 2011
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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