An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries

Abstract

This case is remarkable, clinically and anatomically. REPORT OF CASE H isto ry-K. Z., a girl, aged 16 years, was an elementary school graduate, had gone to business school, and had been employed for eight months pre ceding the illness. There were three other children, two younger and one older; all apparently were perfectly normal. There were no home difficulties, and poverty was not extreme. She had spent September 4 and 5 at Rockaway Beach, where she appeared in perfect health and spirits. She had returned home on the evening of September 5 and slept well. On the morning of September 6, she complained of weakness in the upper extremities and had pain on moving the wrists and elb o w s; she already had marked pallor and was slightly constipated. The symptoms increased in severity until she was admitted to the Beth Israel Hospital, September 15. W hile at home, she had a constant fever, the temperature rising once to 104 F. and staying at other times between 101 and 102. F. Physical Examination.-The patient was a pale girl with "cafe au lait" tinge. A few petechiae were present on the left arm. The lungs and heart revealed nothing abnormal. The spleen and liver were not enlarged. The abdomen was lax and not tender. September 18, the red blood count was 1,330,000; the hemoglobin, 40 per cen t.; the leukocytes, 12,600, of which 65 per cent, were polymorphonuclears. The red cells revealed a central pallor, but there were no nucleated elements. A fragility test showed hemolysis to begin at 0.8, and to be complete at 0.19 (?). No platelet count was made. September 19, the red blood count was 1,120,000; the hemoglobin was 40 per cent., and the leukocytes were 19,000. A blood culture remained sterile. The urine showed marked traces of albumin with hyaline and granular casts. The blood chemistry, September 16, show ed: urea, 21.2 mg. per hundred cubic centim eters; nonprotein nitrogen, 31.25 mg. per hundred cubic centi meters, and creatinin, 1.1 mg. per hundred cubic centimeters. The feces and gastric contents gave a marked reaction for occult blood. Roentgen-ray examination of the chest showed nothing abnormal. The electrocardiogram showed inversion of the T wave in Lead III. The tempera ture, on admission, was 101.8 F. During the week the patient was in the hospital, it ranged between 100 and 102 F. The pulse varied between 100 and 130. The respiration was around 20. The systolic blood pressure was 130, and the diastolic, 60. September 19, there was partial paresis of the left arm and leg; also, a slight facial paralysis. The follow ing day, a double Kernig reflex was noted. That night there was pulmonary edema, which responded to treatment. Soon after, the patient went into com a; respirations became irregular, and she died, September 20. Dr. E. Libman, who saw this patient in consultation, recognized the condition as a new disease.

Chronic relapsing thrombotic thrombocytopenic purpura: three case reports and update of the pathogenesis and therapeutic modalities

“…TTP was ®rst recognized by Moschcowitz in 1924 [18] who described a fulminate and fatal illness in a 16-year-old girl characterized by pallor, petechiae, fever, central nervous system impairment, anaemia and proteinuria. At autopsy, hyaline thrombi were found in terminal arterioles and capillaries.…”

High-Dose Intravenous ?-Immunoglobulin Infusion in the Treatment of Thrombotic Thrombocytopenic Purpura

“…On the second hospital day, plasmapheresis was started and continued for three days. In each plasma exchange, approximately 3 L of plasma was removed and replaced with the Accepted for publication Sept 1,1987 -Schema demonstrating response of platelet counts, hematocnt, lactate dehydrogenase, and reticulocyte counts to different treatment modalities, and improvement after immunoglobulin infusion. same volume of fresh-frozen plasma.…”

Thrombotic microangiopathy in adult-onset Still's disease: case report and review of the literature

“…TTP was originally described by Moschowitz in 1925 as a disease characterized by unique pathological findings of hyaline thrombi in many organs [2]. Amorosi and Ultmann defined the classic pentad of clinical features: thrombocytopenia, MAHA, acute renal impairment, neurologic abnormalities and fever [3].…”