Sickle-cell disease laboratory findings

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aarti Narayan, M.B.B.S [2] Shyam Patel [3]

Overview

The most important laboratory test for sickle cell anemia is a complete blood count (CBC), specifically hemoglobin and hematocrit.

Laboratory findings

Blood tests

Complete blood count (CBC) will reveal haemoglobin levels in the range of 6-8 g/dL with a high reticulocyte count (greater than 1.5%). On a peripheral blood film, one can observe features of hyposplenism i.e., target cells and Howell-Jolly bodies. Sickling of the red blood cells, on a blood film, can be induced by the addition of sodium metabisulphite. Another test is Sickle Solubility test. A mixture of haemoglobin S (Hb S) in a reducing solution e.g., sodium dithionite gives a turbid appearance while normal Hb gives a clear solution. Abnormal hemoglobin forms can be detected on hemoglobin electrophoresis, a form of gel electrophoresis on which the various types of hemoglobin move at varying speed. Sickle-cell hemoglobin (HgbS) and hemoglobin C with sickling (HgbSC)—the two most common forms—can be identified from there. Genetic testing is rarely performed.

For patients with sickle cell disease who present with possible infection, important laboratory testing may include: urinalysis, blood cultures, sputum cultures, and cerebrospinal fluid analysis.