A case is presented of a girl, aged 18, born of parents who were first cousins. She had an atypical retinitis pigmentosa with involvement of the macula. The neurologic examination showed diffuse disease of the central nervous system, as seen in Friedreich’s ataxia. An additional finding (hitherto undescribed) was that of a malformation of the red blood cells. These cells had a peculiar crenated appearance, due to the presence of pseudopods or protoplasmic projections varying in size and shape. They were constantly present in stained blood films taken one year apart. A similar finding in a younger brother, with beginning retinal pigmentary degeneration, added additional proof of the hereditary nature of the condition.
1. The hematologic data in 111 cases of disseminated (systemic) lupus erythematosus are presented.
2. Anemia occurred in practically all patients at some time during their course.
3. Leukopenia was seen initially in about one-third of the patients and developed subsequently in about one-half at some time during their course. Many responded to infection with leukocytosis.
4. Thrombocytopenia occurred its about one-third of the patients on initial counts and developed subsequently in about one-half at some time its their course. Thrombocytopenia was often seen without leukopenia. Marked thrombocytopenia, simulating "idiopathic" thrombocytopenia purpura, was seen in two cases.
5. There was poor correlation between splenomegaly and a reduction in the formed elements of the blood.
6. Bone marrow differential counts were not helpful in making the diagnosis but an increase in plasma cells was frequent.
7. The Hargraves test was an extremely valuable diagnostic aid.
8. Treatment with ACTH and cortisone did not produce any striking hematologic changes.
9. Five cases of unusual interest, including three with hemolytic anemia, were presented.
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