Congenital Hemolytic Anemias Presenting As Severe Anemia in Older Patients

Mild congenital hemolytic anemias can become worse with aging and co-morbidities. We currently care for four patients in whom we established the diagnosis of Hereditary Spherocytosis (HS) or Hereditary Elliptocytosis (HE) after age 70. All had received multiple red blood cell transfusions. One had “failed” hypomethylating agent therapy for misdiagnosed myelodysplastic syndrome. Another patient (after three bone marrow exams) was about to start treatment for multiple myeloma (he had a small monoclonal gammopathy); he now has a normal hemoglobin after splenectomy for HS. The peripheral blood smear was strongly suggestive of the diagnosis in all patients. In three of the four, the diagnosis was confirmed by genetic testing and first-degree relatives were identified who also were affected. In the fourth patient, genetic and family studies are planned. Hematologists and hematopathologists should be vigilant for congenital hemolytic anemias becoming symptomatic in older people, sparing them misdiagnosis and mistreatment. With the de-emphasis on microscopic morphology skills in modern medical curriculae, this may be becoming a larger problem.

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