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Moschcowitz' disease

Alternative eponyms

  • Baehr-Schiffrin disease
  • Baehr-Schiffrin syndrome
  • Moschowitz-Singer-Symmers syndrome
  • Schulman-Upshaw syndrome
  • Upshaw-Schulman syndrome
  • Upshaw factor deficiency syndrome

Related people

A congenital syndrome characterised by thrombocytopenia, purpura, haemolytic anaemia, hyaline thromboses, renal failure and neurological symptoms.

Description

A congenital syndrome characterised by thrombocytopenia, purpura, haemolytic anaemia, hyaline thromboses, renal failure and neurological symptoms. Commonly manifests with jaundice, fever, systemic and retinal haemorrhages, hematuria, confusions, headache, paralysis, paresthesia, convulsions, haemolysis, azotemia, and coma. Both sexes are affected. Prevalent in females; onset from infancy to old age with average age of onset 33 years. Highly fatal within days or weeks.

First reported in 1925 by Moschcowitz, who, on the basis of morphological changes, recognized a previously undescribed entity. Moschcowitz' patient was a girl who presented with an abrupt onset of petechiae and pallor followed rapidly by paralysis, coma, and death. Upon pathologic examination, the small arterioles and capillaries of the patient were found to have thrombi consisting mostly of platelets. Moschowitz hypothesized a "powerful poison which had both agglutinative and hemolytic properties" as the cause of the disease.

In 1936 the disease picture was defined clinically and morphologically and was distinguished differential-diagnostically from other forms of purpura by George Baehr, Paul Klemperer (1887-1964), and Arthur Schiffrin (1904-). Vassar and Spain applied the term «platelet thrombosis syndrome.» Upshaw-Schulman syndrome is a congenital variant.

Bibliography

  • E. Moschcowitz:
    Hyaline thrombosis of the terminal arterioles and capillaries. A hitherto undescribed disease.
    Proceedings of the New York Pathological Society, 1924, 24: 21-24.
  • E. Moschcowitz:
    An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries. An undescribed disease.
    Archives of Internal Medicine, Chicago, 1925, 36: 89-93.
  • G. Baehr, P. Klemperer, A. Schiffrin:
    Acute febrile anemia and thrombocytopenic purpura with diffuse platelet thromboses of capillaries and arterioles.
    Transactions of the Association of American Physicians, Philadelphia, 1936, 51: 43-58.
  • K.Singer, F. P.Bornstein, S. A. Wile:
    Thrombotic cytopenic purpura.Hemorrhagic diathesis with generalized platelet thromboses.
    Blood, New York, 1947, 2: 542- 544.
  • I. Schulman, M. Pierce, A. Lukens, Z. Currimbhoy:
    Studies on thrombopoiesis 1. A factor in normal human plasma required for platelets production; chronic thrombocytopenia due to its deficiency.
    Blood, New York, 1960, 16: 943-957.
  • J. D. Upshaw:
    Congenital deficiency of a factor of normal plasma that reverses microangiopathic hemolysis and thrombocytopenia.
    New England Journal of Medicine, Boston, 1978, 298: 1350-1352.

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