LETTER
Sarcoidosis and Sickle Cell Disease
Gairy Hall and
Jerome Carter
15 January 1993 | Volume 118 Issue 2 | Pages 157-158
TO THE EDITOR:
A few patients with sarcoidosis and hemoglobinopathies have been reported, but only four have been associated with sickle cell disease [1-4]. We describe a patient with sarcoidosis, sickle cell anemia, and persistent thrombocytosis. A 36-year-old black woman with sickle cell anemia was hospitalized with painful sickle cell crisis. Over the preceding months, she had multiple admissions to another hospital because of joint pains, fevers, malaise, and fatigue. Her vital signs and physical examination were normal except for joint tenderness. Her hematocrit was 17%, her platelet count was 1200 x 10 (3/mm)3, and a corrected leukocyte count was 13 x 103/mm3 with a normal differential. Her chest roentgenogram was consistent with pulmonary hypertension. Her painful crisis and fever continued despite transfusions of packed erythrocytes and analgesics. A gallium scan and magnetic resonance imaging showed multifocal nodal disease in the superior mediastinum, retrosternal areas and bilateral pulmonary hila. A right paratracheal lymph node showed a noncaseating granuloma consistent with sarcoidosis with negative fungal and tuberculous cultures. The patient was started on prednisone, and a nonsteroidal antiinflammatory agent, and was discharged.
Sarcoidosis and hemoglobinopathies are two disorders commonly affecting blacks. Sarcoidosis occurs at a rate of about 30 to 40 per 100 000 in the general population, and approximately 0.15% of African Americans are homozygous for sickle cell trait.
The persistent thrombocytosis in this patient may be secondary to her sickle cell disease. Haut and colleagues [1] found a significantly increased mean life span of platelets in persons with sickle cell disease who were not in crisis. Marked thrombocytosis after a crisis suggested increased thrombopoiesis and reticulocytosis [1]. In addition, reactive thrombocytosis has been associated with acute and chronic inflammation.
A study by Greenberg and colleagues [2] showed an 18% prevalence rate of hemoglobinopathies in sarcoid patients, but none had sickle cell disease. Subsequently, Hirchman and Johns [3] showed a 20% prevalence rate that included one patient with sickle cell disease. In 1977, Madigan and colleagues [4] reported a patient with sarcoidosis and sickle cell disease, and in 1981, Young and associates [5] reported the association of the two diseases in three patients, two of whom had sickle cell disease. Physicians should be cognizant of this possible relation.
1. Haut M, Cowan O, Harris J. Platelet function and survival in sickle cell disease. J Lab Clin Med. 1973; 82:44-52.
2. Greenberg S, Atwater J, Isreal H. Frequency of hemoglobinopathies in sarcoidosis. Ann Intern Med. 1965; 62:125-8.
3. Hirschman R, Johns C. Hemoglobin studies in sarcoidosis. Ann Intern Med. 1965; 62:129-32.
4. Madigan J, Gragoudas E, Schwartz P, Lapus J. Peripheral retinal neovascularization in sarcoidosis and sickle cell anemia. Am J Ophthalmol. 1977; 83:387-91.
5. Young R, Castro O, Baxter R, Dunn R, Armstrong E, Cook F, et al. The lung in sickle cell disease: a clinical overview of common vascular, infectious, and other problems. J Nat Med Assoc. 1981; 73:19-26.
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