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Congenital thrombocytopenic purpura
CONGENITAL TttROMBOCYT,0PENIC PURPURA JANET TAL]KADGE; ~/[.~D., AND BENJAMIN BERMAN, ]V[.]). ST. LOUIS, ~V[o.
ONGENITAL thrombocytopenic purpura is a rare condition, but it does
occur often enough so that it must be considered as a possible cause of bleedC ing tendencies in a newborn infant. There have been twenty cases previously reported, and it may be well to review briefly these previous reports before presenting our own cases. Sanford, ~ in 1936, reviewed the literature of twelve cases and presented the ease of a female infant whose mother had also ]lad purpura. Davidson, 2 in 1937, reported thrombocytopenic purpura in an infant whose mother had had a spleneetomy for this condition. Whitney, 3 in 1942, and Urbanski, 4 in 1942, each presented reports of two consecutive children of mothers with purpura. Morrison, ~ in 1945, presented a single case, and Schefrin, 6 in 1945, reported thrombocytopenic purpura in an infant whose mother had never had any bleeding tendencies. We have recently seen thromboeytopenie purpura occur in the neonatal period in three successive offspring of a mother with idiopathic thrombocytopenie purpura treated by spleneetomy three years before the first pregnancy. CASE REPORTS
The mother of these children was first admitted to City Hospital on June 3, 1938, at the age of 17, with menorrhagia, bleeding gmns, and petechiae. The red count at this time was 1,110,000; plate]ets, 1,105; bleeding time, 45 minutes; and clotting time normal, with no clot retractiom Idiopathic thrombocytopenic purpura was diagnosed, and a splenectomy was performed on June 30, 1938, with an uneventful convalescence. Following sp]eneetomy the mother was not seen again until Jan. 17, 1941, when she was seven months pregnant. She had been asymptomatic .until two months previously, but for the past two months had been having frequent nosebleeds. Examination at this time showed the platelet count to be 25,000, the bleeding time 30 minutes. She was followed in prenatal clinic for the next two months with weekly transfusions up to the time of delivery. During this time there was no appreciable change in the number of platelets but there was cessation of bleeding. On March 1, 1941, a full-term female infant, D. H., was born (Chart 1). This baby appeared nomnal a t birth, but six hours after birth petechiae appeared over the body; the platelet count was 8,500; liver and spleen were not palpable. On the fourth day of life, blood appeared in the stools and continued for the next week. On the baby's fifth day, the platelet count had dropped to zero, and it then began to rise spontaneously. Bleeding time was 30 minutes. There was no abnormal bleeding after the tenth day, although the baby received no transfusions until she was 3 weeks old; by this time the platelets had risen spontaneously to 2.0,000: During the next month she received five transfusions, and the platelets continued to increase, showing a precipitous rise at the age of 5 weeks. She was discharged on May 15, 1941, at the age of 2 months, with 542,000 platelets. For the next eighteen months she was followed in clinic, and during this time the blood picture remained normal. The mother was next seen again in prenatal clinic on Jan. 30, 1945, six months pregnant; blood examination showed only three p]atelets per cubic From the Pediatric Service, St. Louis City ttospital. 691
692
THE
JOURNAL
OF
PEDIATRICS
millimeter, although there had been no spontaneous bleeding or peteehiae. She was again followed in clinic with weekly transfusions throughout the remainder of pregnancy mad at delivery had 64;000 platelets, with the bleeding time 15 minutes. A second full-term female infant, C. It., was born on April 24, 1945 (Chart 2). The baby's platelet count immediately after birth was 5,000; six hours later this dropped to zero, bleeding from the cord appeared, and peteehiae were noticed over the body. Liver and spleen was not palpable. On the
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third day, blood appeared in the stools. At the age of one week, the plate]et count remained at zero, bleeding time was 41 minutes, clotting time 15 minutes with no retraction, and blood was still present in the stools. During the second week the platelets began to increase spontaneously, the bleeding time decreasd, and no more bleeding tendencies were apparent. No transfusions were given until the baby was 5 weeks old; by this time the platelet count had risen considerably,
TALMADGE AND BERMAN:
CONGENITAL THROMBOCYTOPENIC
693
PURPURA
and two transfusions were then given because of anemia. This baby was discharged on June 25, 1945, at the age of 2 months, with 981,000 platelets. She was followed in clinic for the next three months, during which time site remained asymptomatic with a normal platelet count, and was seen again at the age of 16 months with no bleeding tendencies and a normal blood picture. u._ E
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The mother's next admission to the hospital was on Aug. 20, 1946, with bleeding gums, petechiae, and 7,580 platelets. She had again been asymptomatic until the last half of pregnancy. A female infant, two weeks premature, was born on Aug. 21, 1946. Thorotrast study of the mother during this admission failed to reveal any accessory spleen. This third child, S. tI. (Chart 3), appeare,d normal at birth, but developed petechiae at the age of 2 days; platelet count was zero, bleeding time 62 minutes, clotting time 3 minutes, with no clot retraction; liver and spleen were not palpable. When the baby was one week old, the platelet count remained zero, the
694
THE
J O U R N A L OF P E D I A T R I C S
bleeding time was still prolonged to 45 minutes with no clot retraction, and blood appeared in the stools. Tibial puncture, performed August 31, was followed by hematoma formation over the puncture site. The baby received transfusions every other day of life beginning on the second day, but there was no change in the number of platelets until she was 2 weeks old. From then on the p]atelets
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gradually increased, with diminishing bleeding time and disappearance of bleedi n g tendencies. She was discharged Oct. 17, 1946, at the age of 2 months, with 169,000 platelets, bleeding time 5 minutes, and clotting time 2 minutes, but still no clot retraction. On Nov. 12, 1946, she had 1,645,000 platelets, and there were no bleeding tendencies apparent. SUMMARY
Congenital thrombocytopenic purpura has occurred as a familial condition in three successive children of a mother previously splenectomized for thrombo-
TAL1VIADGE AI~ID BERMAN:
CONGENITAL THRO~CIBOCYTOPENIC PURPURA
695
cytopenic purpura. In each of these children there has been a gradual spontaneous recovery t o n normal blood picture during the first 2 months of life, and following recovery there has been no recurrence of thrombocytopenia up to the present time. REFERENCES 1. Sanford, H. N. : Leslie, E. I., and Crane, M. M. : Congenital Thrombocytopenia, Am. J. Dis. Child. 51: 1114, 1936. 2. Davidson, L. T..' Congenital Thrombopenia, Am. 2-. Dis. Child. 54: 1324, 1937. 3. Whitney, L: It., and t~arritt, A. S., Jr.: Spontaneous and Hereditary Thrombopenic Purpura in a Mother and Two Sons, Am. J-. Dis. Child. 64: 705, 1942. 4. Urban.ski, A. X.~ and Hutner, C. I.: Thrombopeulc Purpura Complicating Pregnancy, J. A. 1~. A. 120: 754, 1942. 5. Morrison, 1~.~ and Samwick, A . A . : Congenital Essential Thrombopenic Purpura, Am. J. Dis. Child. 70:1151 1945. 6. Shefrin, A. E., and Schechtman~ A.: Congenital Thrombopenia~ Am. J. Dis. Child. 70: 29, 1945.
ONGENITAL thrombocytopenic purpura is a rare condition, but it does
occur often enough so that it must be considered as a possible cause of bleedC ing tendencies in a newborn infant. There have been twenty cases previously reported, and it may be well to review briefly these previous reports before presenting our own cases. Sanford, ~ in 1936, reviewed the literature of twelve cases and presented the ease of a female infant whose mother had also ]lad purpura. Davidson, 2 in 1937, reported thrombocytopenic purpura in an infant whose mother had had a spleneetomy for this condition. Whitney, 3 in 1942, and Urbanski, 4 in 1942, each presented reports of two consecutive children of mothers with purpura. Morrison, ~ in 1945, presented a single case, and Schefrin, 6 in 1945, reported thrombocytopenic purpura in an infant whose mother had never had any bleeding tendencies. We have recently seen thromboeytopenie purpura occur in the neonatal period in three successive offspring of a mother with idiopathic thrombocytopenie purpura treated by spleneetomy three years before the first pregnancy. CASE REPORTS
The mother of these children was first admitted to City Hospital on June 3, 1938, at the age of 17, with menorrhagia, bleeding gmns, and petechiae. The red count at this time was 1,110,000; plate]ets, 1,105; bleeding time, 45 minutes; and clotting time normal, with no clot retractiom Idiopathic thrombocytopenic purpura was diagnosed, and a splenectomy was performed on June 30, 1938, with an uneventful convalescence. Following sp]eneetomy the mother was not seen again until Jan. 17, 1941, when she was seven months pregnant. She had been asymptomatic .until two months previously, but for the past two months had been having frequent nosebleeds. Examination at this time showed the platelet count to be 25,000, the bleeding time 30 minutes. She was followed in prenatal clinic for the next two months with weekly transfusions up to the time of delivery. During this time there was no appreciable change in the number of platelets but there was cessation of bleeding. On March 1, 1941, a full-term female infant, D. H., was born (Chart 1). This baby appeared nomnal a t birth, but six hours after birth petechiae appeared over the body; the platelet count was 8,500; liver and spleen were not palpable. On the fourth day of life, blood appeared in the stools and continued for the next week. On the baby's fifth day, the platelet count had dropped to zero, and it then began to rise spontaneously. Bleeding time was 30 minutes. There was no abnormal bleeding after the tenth day, although the baby received no transfusions until she was 3 weeks old; by this time the platelets had risen spontaneously to 2.0,000: During the next month she received five transfusions, and the platelets continued to increase, showing a precipitous rise at the age of 5 weeks. She was discharged on May 15, 1941, at the age of 2 months, with 542,000 platelets. For the next eighteen months she was followed in clinic, and during this time the blood picture remained normal. The mother was next seen again in prenatal clinic on Jan. 30, 1945, six months pregnant; blood examination showed only three p]atelets per cubic From the Pediatric Service, St. Louis City ttospital. 691
692
THE
JOURNAL
OF
PEDIATRICS
millimeter, although there had been no spontaneous bleeding or peteehiae. She was again followed in clinic with weekly transfusions throughout the remainder of pregnancy mad at delivery had 64;000 platelets, with the bleeding time 15 minutes. A second full-term female infant, C. It., was born on April 24, 1945 (Chart 2). The baby's platelet count immediately after birth was 5,000; six hours later this dropped to zero, bleeding from the cord appeared, and peteehiae were noticed over the body. Liver and spleen was not palpable. On the
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I B
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I II
I 12
30 M~n.
"lo'J-'l';nq Time Blood Ioo. Trons ~usaon
Chart
I.--D.
H., March
to May,
1941.
third day, blood appeared in the stools. At the age of one week, the plate]et count remained at zero, bleeding time was 41 minutes, clotting time 15 minutes with no retraction, and blood was still present in the stools. During the second week the platelets began to increase spontaneously, the bleeding time decreasd, and no more bleeding tendencies were apparent. No transfusions were given until the baby was 5 weeks old; by this time the platelet count had risen considerably,
TALMADGE AND BERMAN:
CONGENITAL THROMBOCYTOPENIC
693
PURPURA
and two transfusions were then given because of anemia. This baby was discharged on June 25, 1945, at the age of 2 months, with 981,000 platelets. She was followed in clinic for the next three months, during which time site remained asymptomatic with a normal platelet count, and was seen again at the age of 16 months with no bleeding tendencies and a normal blood picture. u._ E
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Tro,ls~us;on C h a r t 2 . - - C . ~I., A p r i l to July, 1945.
The mother's next admission to the hospital was on Aug. 20, 1946, with bleeding gums, petechiae, and 7,580 platelets. She had again been asymptomatic until the last half of pregnancy. A female infant, two weeks premature, was born on Aug. 21, 1946. Thorotrast study of the mother during this admission failed to reveal any accessory spleen. This third child, S. tI. (Chart 3), appeare,d normal at birth, but developed petechiae at the age of 2 days; platelet count was zero, bleeding time 62 minutes, clotting time 3 minutes, with no clot retraction; liver and spleen were not palpable. When the baby was one week old, the platelet count remained zero, the
694
THE
J O U R N A L OF P E D I A T R I C S
bleeding time was still prolonged to 45 minutes with no clot retraction, and blood appeared in the stools. Tibial puncture, performed August 31, was followed by hematoma formation over the puncture site. The baby received transfusions every other day of life beginning on the second day, but there was no change in the number of platelets until she was 2 weeks old. From then on the p]atelets
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Blood Ioocc Trons'~usion
C h a r t 3 . - - S , H . , A u g u s t to N o v e m b e r , 1946.
gradually increased, with diminishing bleeding time and disappearance of bleedi n g tendencies. She was discharged Oct. 17, 1946, at the age of 2 months, with 169,000 platelets, bleeding time 5 minutes, and clotting time 2 minutes, but still no clot retraction. On Nov. 12, 1946, she had 1,645,000 platelets, and there were no bleeding tendencies apparent. SUMMARY
Congenital thrombocytopenic purpura has occurred as a familial condition in three successive children of a mother previously splenectomized for thrombo-
TAL1VIADGE AI~ID BERMAN:
CONGENITAL THRO~CIBOCYTOPENIC PURPURA
695
cytopenic purpura. In each of these children there has been a gradual spontaneous recovery t o n normal blood picture during the first 2 months of life, and following recovery there has been no recurrence of thrombocytopenia up to the present time. REFERENCES 1. Sanford, H. N. : Leslie, E. I., and Crane, M. M. : Congenital Thrombocytopenia, Am. J. Dis. Child. 51: 1114, 1936. 2. Davidson, L. T..' Congenital Thrombopenia, Am. 2-. Dis. Child. 54: 1324, 1937. 3. Whitney, L: It., and t~arritt, A. S., Jr.: Spontaneous and Hereditary Thrombopenic Purpura in a Mother and Two Sons, Am. J-. Dis. Child. 64: 705, 1942. 4. Urban.ski, A. X.~ and Hutner, C. I.: Thrombopeulc Purpura Complicating Pregnancy, J. A. 1~. A. 120: 754, 1942. 5. Morrison, 1~.~ and Samwick, A . A . : Congenital Essential Thrombopenic Purpura, Am. J. Dis. Child. 70:1151 1945. 6. Shefrin, A. E., and Schechtman~ A.: Congenital Thrombopenia~ Am. J. Dis. Child. 70: 29, 1945.