Intraerythrocytic Hemoglobin Crystals in Sickle Cell-Hemoglobin C Disease

Abstract

On 70 per cent of the blood smears from 60 cases of electrophoretically proven sickle cell-hemoglobin C disease, there is observed a misshapen erythrocyte that contains condensed hemoglobin crystals which are dark-hued, homogeneous and elongated and which have parallel sides with one end terminating in a pyramid or rounded shape. A red blood corpuscle may have multiple protuberances at varying angles to each other. The incidence of intracellular hemoglobin crystals was found to be 0-24 per 1000 red blood cells with an average of 3.2/1000. Recognition of this unusual morphology is presumptive evidence of sickle cell-hemoglobin C and warrants examination by electrophoretic procedures.

Diagnosis from the Blood Smear

“…Patients with sickle cell anemia (in which there is homozygosity for hemoglobin S) have anemia, but those with compound heterozygosity for hemoglobin S and hemoglobin C may have a normal hemoglobin level, and the condition thus may be confused with sickle cell trait if a blood smear is not examined. Consideration of the blood-smear features, of the hemoglobin level, and of the results of a sickle cell solubility test usually permits an ac-curate diagnosis 4,5 (Fig. 4B and 4C).…”

Red blood cell morphology

“…Patients with SC disease and any of the sickle thalassemia compound disorders (including Sβ 0 and SS‐α thalassemia) may have considerably more target cells than patients with uncomplicated SS. Patients with SC disease may also demonstrate C crystals in some RBCs . C crystals and targets by themselves, without sickle cells, of course may suggest homozygosity for hemoglobin C. Spherocytes have two common causes: immune‐mediated hemolysis and hereditary spherocytosis (HS).…”

Acute liver function decompensation in a patient with sickle cell disease managed with exchange transfusion and endoscopic retrograde cholangiography

“…Infrequently, levels of transaminases may exceed 1000 mg/dl, which may indicate severe hepatic hypoxia. Conservative management is the usual approach and almost all cases resolve spontaneously after a couple of weeks [Diggs and Bell, 1965;Rosenblate et al 1970;Sheehy, 1977;Schubert, 1986;Friedman, 2007]. Patients with SCIC present with remarkable clinical features such as extreme hyperbilirubinemia, with a mixed conjugated and unconjugated pattern and jaundice with hepatomegaly, coagulopathy and renal insufficiency in the absence of other acute liver disease.…”