Letters and Correspondence
Successful Treatment of Thrombotic Thrombocytopenic Purpura With High-Dose Corticosteroid To the Editor: The acute or fulminant feature of thrombotic thrombocytopenic purpura (TTP) usually requires multiple treatments including infusion of fresh frozen plasma (FFP), plasma exchange, and corticosteroid immediately after the establishment of diagnosis [ 1,2]. Multiple forms of treatments, however, induce difficulties in evaluation of effectiveness of each treatment. When the patient receives each treatment independently, it becomes possible to assess effectiveness. A 54-year-old woman, who was admitted because of fever, transient right hemiparesis, anemia, and thrombocytopenia. was diagnosed as hav-
dipyridamole 300 infusion of FFP
mPSL prednisolone 60 50 40 30 25 20
15
....... -250
1000 plasma exchange
3000ml/day
I
69
ing TTP. One month before admission, the patient first noticed purpura on extremities. Laboratory data included a hemoglobin of 8.0 g/IOO ml, hematocrit 24.8%, red blood cell 2,460,0001p1, reticulocyte 21.4%, platelet 13,000lp1, leukocyte 7,2OO/pl, 14 erythroblasts/lOO leukocytes, total bilirubin 1.4 mg/100 ml, serum lactate dehydrogenase (LDH) 1,446 U, and serum creatinine 0.8 mgllOO ml. Infusion of FFP was started with dipyridamole, but platelets did not increase in number (Fig. I). The subsequent plasma exchange also failed. Hemoglobin gradually decreased to 5.3 g/IOO ml. Pulse therapy with methylprednisolone (mPSL) 1,000 mg/day was performed and was replaced by prednisolone 60 mg/day. Platelets gradually increased in number up to 156,00O/pI within 2 weeks after mPSL therapy, accompanying the elevation of hemoglobin to 9.7 g/IOO ml. A high LDH was significantly lowered by mPSL. The percentage of fragmented red blood cells examined with differential interference microscopy was 46.4% on the second day of mPSL therapy. It rapidly decreased to 30.2% the next day, followed by a gradual decrease to less than 1.O%. The change of fragmented red blood cells was positively associated with the LDH level. The dose of prednisolone was gradually reduced. After 8-month follow-up, the maintenance dose of prednisolone is 7.5 mgiday and hemoglobin and platelets are 13.9 g/IOO ml and 365,0001p1, respectively. Transfusion of red blood cells and platelets was not carried out during the clinical course. Slow progression and mild feature of our case allowed us a step by step treatment and to assess the effectiveness of each therapy. In plasma exchange, FFP should be used until LDH decreases to below 700 U [3]. In our case, the LDH level was once decreased to 576 U by plasma exchange, then the level was markedly increased to more than 2,000 U. The marked increase in LDH level forced us to resign the plasma therapy. The rapid decrease in the percentage of fragmented red blood cells and the parallel fall of the LDH level during mPSL therapy clearly indicated the significant effectiveness of this therapy. It is generally accepted that plasma therapy is indispensable and the usefulness of corticosteroid is not fully defined [ 1,2]. A few cases, however, responded to corticosteroid alone [1,2,4]. In addition, a recent report also suggested the possibility that TTP is controllable by high-dose intravenous mPSL therapy. In this case, however, high-dose corticosteroid was chosen in the initial therapy [5].Therefore, it is not clear whether or not plasma therapy was effective. The present report is the first to demonstrate the clinical effectiveness of mPSL therapy against TTP which did not respond to treatments with FFP infusion and plasma exchange.
MICHIHIRO TOYOSHIGE YUZURU ZAITSU OKAFUJI KOICHIRO YASUSHI INOUE YUKIOHIROSHIGE NOBORU MATSUYOTO KOHEI KAKU TOSHIO KANEKO
@
Third Department of Internal Medicine, Yamaguchi University School of Medicine, Ube, Japan
REFERENCES
Fig. 1. Clinical course after admission. The doses of dipyridamole, mPSL, and prednisolonewere indicated as mg/day. FFP was infused 8 Ulday for 8 days. Plasma exchange 3,000 mllday was performed for 2 days. Fragmentationof red blood cells (%): ratio of fragmented red blood cells in total red blood cells. mPSL, methylprednisolone; LDH, serum lactate dehydrogenase; FFP, fresh frozen plasma.
1 . Eyrnes JJ, Lian ECY: Recent therapeutic advances in thrombotic thrombocytopenic purpura. Semin Thromb Hemost 5:199, 1979. 2 . Eukowski RM, Hewlett JS, Reimer RR, Groppe CW, Weick JK, Livingston RB: Therapy of thrombotic thrombocytopenic purpura: An overview. Semin Thromb Hemost7:1, 1981. 3. Taft EG: Thrombotic thronibocytopenic purpura and dose of plasma exchange. Blood 54:842, 1979. 4. Petitt RM: Thrombotic thrombocytopenic purpura: A thirty year review. Semin Thromb Hemost 6:350,1980. 5. ozsoylu S: High-dose intravenous methylprednisolone for thrombotic thrombocytopenic purpura. Acta Hematol 84: 110, 1990.
Successful Treatment of Thrombotic Thrombocytopenic Purpura With High-Dose Corticosteroid To the Editor: The acute or fulminant feature of thrombotic thrombocytopenic purpura (TTP) usually requires multiple treatments including infusion of fresh frozen plasma (FFP), plasma exchange, and corticosteroid immediately after the establishment of diagnosis [ 1,2]. Multiple forms of treatments, however, induce difficulties in evaluation of effectiveness of each treatment. When the patient receives each treatment independently, it becomes possible to assess effectiveness. A 54-year-old woman, who was admitted because of fever, transient right hemiparesis, anemia, and thrombocytopenia. was diagnosed as hav-
dipyridamole 300 infusion of FFP
mPSL prednisolone 60 50 40 30 25 20
15
....... -250
1000 plasma exchange
3000ml/day
I
69
ing TTP. One month before admission, the patient first noticed purpura on extremities. Laboratory data included a hemoglobin of 8.0 g/IOO ml, hematocrit 24.8%, red blood cell 2,460,0001p1, reticulocyte 21.4%, platelet 13,000lp1, leukocyte 7,2OO/pl, 14 erythroblasts/lOO leukocytes, total bilirubin 1.4 mg/100 ml, serum lactate dehydrogenase (LDH) 1,446 U, and serum creatinine 0.8 mgllOO ml. Infusion of FFP was started with dipyridamole, but platelets did not increase in number (Fig. I). The subsequent plasma exchange also failed. Hemoglobin gradually decreased to 5.3 g/IOO ml. Pulse therapy with methylprednisolone (mPSL) 1,000 mg/day was performed and was replaced by prednisolone 60 mg/day. Platelets gradually increased in number up to 156,00O/pI within 2 weeks after mPSL therapy, accompanying the elevation of hemoglobin to 9.7 g/IOO ml. A high LDH was significantly lowered by mPSL. The percentage of fragmented red blood cells examined with differential interference microscopy was 46.4% on the second day of mPSL therapy. It rapidly decreased to 30.2% the next day, followed by a gradual decrease to less than 1.O%. The change of fragmented red blood cells was positively associated with the LDH level. The dose of prednisolone was gradually reduced. After 8-month follow-up, the maintenance dose of prednisolone is 7.5 mgiday and hemoglobin and platelets are 13.9 g/IOO ml and 365,0001p1, respectively. Transfusion of red blood cells and platelets was not carried out during the clinical course. Slow progression and mild feature of our case allowed us a step by step treatment and to assess the effectiveness of each therapy. In plasma exchange, FFP should be used until LDH decreases to below 700 U [3]. In our case, the LDH level was once decreased to 576 U by plasma exchange, then the level was markedly increased to more than 2,000 U. The marked increase in LDH level forced us to resign the plasma therapy. The rapid decrease in the percentage of fragmented red blood cells and the parallel fall of the LDH level during mPSL therapy clearly indicated the significant effectiveness of this therapy. It is generally accepted that plasma therapy is indispensable and the usefulness of corticosteroid is not fully defined [ 1,2]. A few cases, however, responded to corticosteroid alone [1,2,4]. In addition, a recent report also suggested the possibility that TTP is controllable by high-dose intravenous mPSL therapy. In this case, however, high-dose corticosteroid was chosen in the initial therapy [5].Therefore, it is not clear whether or not plasma therapy was effective. The present report is the first to demonstrate the clinical effectiveness of mPSL therapy against TTP which did not respond to treatments with FFP infusion and plasma exchange.
MICHIHIRO TOYOSHIGE YUZURU ZAITSU OKAFUJI KOICHIRO YASUSHI INOUE YUKIOHIROSHIGE NOBORU MATSUYOTO KOHEI KAKU TOSHIO KANEKO
@
Third Department of Internal Medicine, Yamaguchi University School of Medicine, Ube, Japan
REFERENCES
Fig. 1. Clinical course after admission. The doses of dipyridamole, mPSL, and prednisolonewere indicated as mg/day. FFP was infused 8 Ulday for 8 days. Plasma exchange 3,000 mllday was performed for 2 days. Fragmentationof red blood cells (%): ratio of fragmented red blood cells in total red blood cells. mPSL, methylprednisolone; LDH, serum lactate dehydrogenase; FFP, fresh frozen plasma.
1 . Eyrnes JJ, Lian ECY: Recent therapeutic advances in thrombotic thrombocytopenic purpura. Semin Thromb Hemost 5:199, 1979. 2 . Eukowski RM, Hewlett JS, Reimer RR, Groppe CW, Weick JK, Livingston RB: Therapy of thrombotic thrombocytopenic purpura: An overview. Semin Thromb Hemost7:1, 1981. 3. Taft EG: Thrombotic thronibocytopenic purpura and dose of plasma exchange. Blood 54:842, 1979. 4. Petitt RM: Thrombotic thrombocytopenic purpura: A thirty year review. Semin Thromb Hemost 6:350,1980. 5. ozsoylu S: High-dose intravenous methylprednisolone for thrombotic thrombocytopenic purpura. Acta Hematol 84: 110, 1990.
