blood Sickle Cell Disease Articles

Decreased median survival of adults with sickle cell disease after adjusting for left truncation bias: a pooled analysis
Michael R. DeBaun, Djamila L. Ghafuri, Mark Rodeghier, Poulami Maitra, Shruti Chaturvedi, Adetola Kassim, Kenneth I. Ataga
Feb 07, 2019
A phase 1/2 ascending dose study and open-label extension study of voxelotor in patients with sickle cell disease
Jo Howard, Claire Jane Hemmaway, Paul Telfer, D. Mark Layton, John Porter, Moji Awogbade, Timothy Mant, Daniel D. Gretler, Kobina Dufu, Athiwat Hutchaleelaha, Mira Patel, Vincent Siu, Sandra Dixon, Noel Landsman, Margaret Tonda, Joshua Lehrer-Graiwer
Jan 17, 2019
Resolution of sickle cell disease–associated inflammation and tissue damage with 17R-resolvin D1
Alessandro Matte, Antonio Recchiuti, Enrica Federti, Bérengère Koehl, Thomas Mintz, Wassim El Nemer, Pierre-Louis Tharaux, Valentine Brousse, Immacolata Andolfo, Alessia Lamolinara, Olga Weinberg, Angela Siciliano, Paul C. Norris, Ian R. Riley, Achille Iolascon, Charles N. Serhan, Carlo Brugnara, Lucia De Franceschi
Jan 17, 2019
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
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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 early-relapsing follicular lymphoma
Carla Casulo, Paul M. Barr
Apr 04, 2019
How I Treat Chronic Myeloid Leukemia in Children and Adolescents
Nobuko Hijiya, Meinolf Suttorp
Mar 27, 2019
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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