Thrombotic Thrombocytopenic Purpura

Abstract

In 1954, Singer reviewed the subject of thrombotic thrombocytopenic purpura, collecting 55 cases from the literature.1 Wile and Sturgeon, in 1956, compiled an additional 19 instances and added 3 case reports in children.2 We are aware of an additional 25 reports during the years 1954-1957, bringing the total number of cases to over 100 In general the clinical course of this disorder has been most unfavorable, the majority of patients dying within eight weeks of the recognized onset of the disease.In rare instances survival for more than one year has been reported.23,24,26,28,29 Several of these patients had atypical manifestations, so that it is difficult to ascertain the time of onset of the disease and the survival time in all of these cases.No well-documented typical case has undergone long-term spontaneous remission, and a variety of therapeutic endeavors has in general failed to influence the course of the disease. There have been no prolonged remissions reported following the use of corticotropin (ACTH) or steroids alone, but in four instances temporary improvement did take place, lasting for 36 days in one patient10 and for 13 days in another.28 The other two patients received steroids, im¬ proved, and were then splenectomized.26,29Only one remission longer than one year has followed splenectomy alone.28Four patients have remitted following treatment with both steroids and splenec¬ tomy.23·24'26·29 On the other hand, splenec¬ tomy and steroids or corticotropin alone or in combination have apparently failed in most reported cases (e. g., references 1-7, 10, [15][16][17][18][19][20][21]25,[27][28][29][30][31][32][33][34][35]. In most instances the amount of steroid employed was not large, and frequently the use of these drugs was restricted to the last few days of life. The present report documents two cases with long-term and continuing remission follow¬ ing the use of large amounts of steroids. Report of CasesCase 1.-A 26-year-old white farmer was ad¬ mitted to the Vanderbilt University Hospital in a confused and disoriented state on June 17, 1955. Three years prior to admission he experienced tem¬ porary loss of consciousness but no other obvious sequelae following an automobile accident. From 1953 to the time of admission there were several attacks of syncope. Otherwise the patient had been in excellent health until the sudden onset of frontal headache, "tingling" paresthesias of the right arm, and weakness of the right side of the body three weeks prior to admission. Within 24 hours he became asymptomatic and returned to work.Ten days before admission there had occurred an episode of dysarthria, confusion, numbness of the face, weakness of the extremities, and vomiting of a small amount of blood. These manifestations lasted but 12 hours, and the patient continued to work despite subsequent repeated occurrence of acroparesthesias lasting 1-60 minutes. His associ¬ ates noted "yellow eyes." Two days before ad¬ mission there was another attack of confusion, "tremors all over," dysarthria, numbness and weak...

Early diagnosis of thrombotic microangiopathy by paraffin sections of aspirated bone-marrow.

“…Successful diagnosis by marrow aspiration was also achieved in one patient by Wile and Sturgeon (1956), and by marrow trephine in the case reported by Morey et al (1956). On the other hand, sections of aspirated or trephined bone marrow failed to show the thrombotic lesions in single cases reported by Singer, Motulsky, and Shanberge (1950), Green and Green (1953), Burke and Hartmann (1959), and in the case reported by Ritz, Groisser, and Banowitch (1956) in which, nevertheless, the lesions were found in the marrow sections at necropsy.…”

“…Since Bernheim (1943) necropsy has usually been devoid of the specific lesions, as in 13 cases mentioned by Symmers (1956), though it gave a positive result in one of Symmers's own cases, and in one of Burke and Hartmann's (1959) two cases. In the other two fatal cases of thrombotic microangiopathy reported by Symmers the characteristic histological changes were present in the uterine curettings and appendix, respectively, which had been obtained surgically before the disease had developed or the diagnosis had been suspected.…”

“…In a number of cases splenectomy has been performed as a therapeutic measure, and most spleens have shown the diagnostic lesion (reviewed by Symmers, 1956). Skin biopsies have usually been negative, as in cases reported by Adelson and Stefanini (1952), Morey, White, and Daily (1956), Stuart and MacGregor-Robertson (1956), Burke and Hartmann (1959), and Griffiths and Irving (1961); though positive results were obtained in the case reported by Beigelman (1951) and in one of the five cases mentioned by Symmers (1956).…”

Generalized Cytomegalic Inclusion-Body Disease Associated With Pneumocystis Pneumonia in Adults

“…Although the number of acceptable published cases of thrombotic purpura is now over 150, the clinicopathological picture defined in the earlier studies has not been much amplified. The diagnosis is now made during life in a higher proportion of cases, and, although treatment is unavailing in the great majority of cases, large doses of steroids (with or without splenectomy) have been followed by long-lasting remissions in a few cases (Burke and Hartmann, 1959), and there is therefore some hope that the hitherto uniformly mortal outcome may at least cpccasionally be avoided. This might have been so in the case described here, in view of the remarkable remission, which, however, was cut short by the pulmonary embolism.…”

Thrombotic Thrombocytopenic Purpura

“…The early use of steroids and A.C.T.H. may be beneficial (Burke and Hartmann, 1959). The main clinical manifestations are fever, haemolytic anaemia, thrombocytopenia, purpura, and involvement of various organs with the relative exclusion of the lungs; the central nervous system and the kidneys are most often involved, but the myocardium (Frick and Hitzig, 1959), gastrointestinal tract (Hellstrom, Nash, andFisher, 1959), spleen (Gore, 1950), lymph nodes (Beigelman, 1951), and other organs are occasionally also clinically involved.…”