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Washington University Experience | MISCELLANEOUS | Familial erythrophagic lymphohistiocytosis | 2A0 History - Case AANP DSS 1980, case 2
Case 2 History (AANP Diagnostic Slide Session, 1980, Case 2) ---- The patient was an 11 month old female who developed nose and gingival bleeding of one week's duration. The child was well until she fell out of bed. Aside from a scalp bruise, there were no problems until 3 weeks later, when she developed hypersomnia and vomiting. Also at about this time there began a gradual decline in the child's previously normal developmental abilities. She was felt to have had a concussion, and an anemia was discovered for which iron and vitamins were prescribed. The mother had had some type of anemia as a child, from which she had fully recovered. ---- Past medical history was otherwise unremarkable. On admission, physical examination revealed massive splenomegaly and multiple cranial nerve palsies. Pertinent laboratory findings included WBC 1700 with 2% segs., 3% bands, 89% lymphocytes, 4% monocytes, 1% metamyelocytes, 1% myelocytes and 8,000 platelets. Rgb 6.2 gm., Rct 20%, reticulocyte count 12.2. She developed a right hemiplegia. Bone marrow examination was consistent with severe peripheral destruction of blood elements. EEG showed diffuse marked slowing. Because of her hypersplenism, splenectomy was performed. Liver biopsy showed massive fatty change. She developed choreo-athetoid movements and cortical blindness. Chemotherapy was begun 4 days prior to death; however, she further deteriorated and died. ----
