Short Communication Acia haemal. 62: 282-284 (1979)
Acute Promyelocytic Leukemia in Childhood Report of a Case with a Review of the Literature Dan Engelhard, Shaul Yatziv, Eliezer A. Rachmilewitz and Aaron Polliack Departments of Pediatrics and Hematology, Hadassah University Hospital and Hebrew University-Hadassah Medical School, Jerusalem
Key Words.. Acute leukemia • Childhood leukemia • DIC • Promyelocyte Abstract. A rare case of acute promyelocytic leukemia (APL) is reported in a 7-vearold boy. The patient displayed the typical features of APL including impaction of the marrow with promyelocytes, marked elevation of the serum vitamin B12 and transcobalamin I levels and a hemorrhagic diathesis. The bleeding diathesis in this case was due to thrombocytopenia, and there was no evidence for disseminated intravascular coagulation.
Case Report A 7-year-old Arab boy was admitted to the Hadassah University Hospital, Jerusalem, after 2 days o f m elena and gingiva) bleeding
On admission, physical examination revealed a pale, extremely ill child with a fever of 39 °C and severe melena. There were no ecchymoses of pur pura. Laboratory investigations revealed severe anemia with a hemoglobin of 2.6 g°/o. The white cell count was 5,200/mm3 with 58% promyelo cytes and the platelet count was 4,000/mm3. All other coagulation studies were within normal lim its. Serum vitamin B12 and Bl2 binding proteins were grossly abnormal: serum vitamin B12 was 3,000 pg/ml, unsaturated vitamin B12 binding ca pacity 10,300 pg/ml, transcobalamin I 9,579 pg/ ml, transcobalamin II 721 pg/ml, and transcoba-
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Acute promyelocytic leukemia (APL) is a rare variant of acute myeloblastic leuke mia (AML) and constitutes approximately 3-5°/o of all acute leukemias [1, 2], APL is rare in childhood, and of more than 125 cases reported until now, only 5 have been children under the age of 10 years [1, 3, 4]. In a review of close to 2,000 cases of acute childhood leukemia [5], APL was not even recognized as a separate entity. During the past 5 years we have treated over 100 cases of children with acute leuke mia and the patient reported here is the first case of APL that we have encountered. This patient had classical APL with a bleeding diathesis due to severe thrombocytopenia.
283
Acute Promyelocytic Leukemia in Childhood
Fig. 1. Bone marrow smear showing many hypergranular promyelocytes. X 1, 100.
Discussion In nearly all cases of APL, it is the bleed ing tendency that initially causes the patient to seek medical attention. The bleeding dia thesis is generally more severe, than the bleeding encountered in other acute leuke mias because disseminated intravascular coagulation (DIC) is present in most pa tients [3], However, in our patient the
bleeding was due to thrombocytopenia and there was no evidence for DIC. In the absence of liver damage, the de termination of serum vitamin BI2 concen trations and levels of vitamin B12 binding proteins have been shown to be both reli able and useful aids in the diagnosis of APL [4]. Our patient did not achieve remission and died of septicemia 3 weeks after the di agnosis of APL was made. Since 1967, with the introduction of daunorubicin, APL has become a treatable disease and complete re mission can be achieved in over half the pa tients with a median survival of 26 months |1J. However, as in our case, the cause of death in patients not in remission is invaria bly sepsis or massive hemorrhage.
References 1 Bernard, J.; Weil, M.; Boiron, M.; Jacquillat, C.; Flandrin, G., and Gemon, M. F.: Acute promyelocytic leukemia. Result of treatment of daunorubicin. Blood 41: 489-496 (1973). 2 Flandrin, G. and Bernard, J.: Cytolog'cal clas
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lamin III zero. Liver function tests were all within normal limits. The bone marrow was impacted with imma ture cells and aspiration resulted in a dry tap. A trephine biopsy was performed and histopathology and touch imprints of the biopsy revealed mas sive infiltration with promyelocytes (fig. 1), some of which contained Auer rods. Treatment with daunomycin was started, plate let transfusions were administered. Nevertheless, severe thrombocytopenia persisted and massive gastrointestinal bleeding continued constantly throughout the hospitalization. Except for persis tent thrombocytopenia, repeated coagulation stud ies were all within normal limits, and there was no laboratory evidence of consumption coagulopathy. On the 15th day of hospitalization, the patient developed septicemia and died.
sification of acute leukemias: a survey of 1,400 cases. Blood Cells /; 7-15 (1975). 3 Gralnick, H. R.; Bargley, J., and Abrell, E.: Heparin treatment for the hemorrhagic dia thesis of acute promyelocytic leukemia. Am. J. Med. 52: 167-174 (1972). 4 Rachmilewitz, D.; Rachmilewitz, E. A.; Polliack, A., and Hcrshko, C.: Acute promyelocy tic leukemia: a report of five cases with a com ment on the diagnostic significance of serum vitamin B12 determination. Br. J. Haemat. 22: 87-92 (1972). 5 Pierce, J. I.; Borges, W. H.; Hcyn, R.; Wolff, J.
Engel hard/Yatziv/Rachmilewitz/Pol hack
A., and Gilbert, E. S.: Epidemiological factors and survival experience in 1,770 children with acute leukemia. Cancer 23: 1296-1304 (1969).
Received: February 8, 1979 Accepted: May 29, 1979 Prof. Aaron Pol hack, MD. Department of Hematology, Hadassah University Hospital, Jerusalem (Israel)
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284
Acute Promyelocytic Leukemia in Childhood Report of a Case with a Review of the Literature Dan Engelhard, Shaul Yatziv, Eliezer A. Rachmilewitz and Aaron Polliack Departments of Pediatrics and Hematology, Hadassah University Hospital and Hebrew University-Hadassah Medical School, Jerusalem
Key Words.. Acute leukemia • Childhood leukemia • DIC • Promyelocyte Abstract. A rare case of acute promyelocytic leukemia (APL) is reported in a 7-vearold boy. The patient displayed the typical features of APL including impaction of the marrow with promyelocytes, marked elevation of the serum vitamin B12 and transcobalamin I levels and a hemorrhagic diathesis. The bleeding diathesis in this case was due to thrombocytopenia, and there was no evidence for disseminated intravascular coagulation.
Case Report A 7-year-old Arab boy was admitted to the Hadassah University Hospital, Jerusalem, after 2 days o f m elena and gingiva) bleeding
On admission, physical examination revealed a pale, extremely ill child with a fever of 39 °C and severe melena. There were no ecchymoses of pur pura. Laboratory investigations revealed severe anemia with a hemoglobin of 2.6 g°/o. The white cell count was 5,200/mm3 with 58% promyelo cytes and the platelet count was 4,000/mm3. All other coagulation studies were within normal lim its. Serum vitamin B12 and Bl2 binding proteins were grossly abnormal: serum vitamin B12 was 3,000 pg/ml, unsaturated vitamin B12 binding ca pacity 10,300 pg/ml, transcobalamin I 9,579 pg/ ml, transcobalamin II 721 pg/ml, and transcoba-
Downloaded by: Stockholm University Library 130.237.165.40 - 12/5/2017 12:36:50 PM
Acute promyelocytic leukemia (APL) is a rare variant of acute myeloblastic leuke mia (AML) and constitutes approximately 3-5°/o of all acute leukemias [1, 2], APL is rare in childhood, and of more than 125 cases reported until now, only 5 have been children under the age of 10 years [1, 3, 4]. In a review of close to 2,000 cases of acute childhood leukemia [5], APL was not even recognized as a separate entity. During the past 5 years we have treated over 100 cases of children with acute leuke mia and the patient reported here is the first case of APL that we have encountered. This patient had classical APL with a bleeding diathesis due to severe thrombocytopenia.
283
Acute Promyelocytic Leukemia in Childhood
Fig. 1. Bone marrow smear showing many hypergranular promyelocytes. X 1, 100.
Discussion In nearly all cases of APL, it is the bleed ing tendency that initially causes the patient to seek medical attention. The bleeding dia thesis is generally more severe, than the bleeding encountered in other acute leuke mias because disseminated intravascular coagulation (DIC) is present in most pa tients [3], However, in our patient the
bleeding was due to thrombocytopenia and there was no evidence for DIC. In the absence of liver damage, the de termination of serum vitamin BI2 concen trations and levels of vitamin B12 binding proteins have been shown to be both reli able and useful aids in the diagnosis of APL [4]. Our patient did not achieve remission and died of septicemia 3 weeks after the di agnosis of APL was made. Since 1967, with the introduction of daunorubicin, APL has become a treatable disease and complete re mission can be achieved in over half the pa tients with a median survival of 26 months |1J. However, as in our case, the cause of death in patients not in remission is invaria bly sepsis or massive hemorrhage.
References 1 Bernard, J.; Weil, M.; Boiron, M.; Jacquillat, C.; Flandrin, G., and Gemon, M. F.: Acute promyelocytic leukemia. Result of treatment of daunorubicin. Blood 41: 489-496 (1973). 2 Flandrin, G. and Bernard, J.: Cytolog'cal clas
Downloaded by: Stockholm University Library 130.237.165.40 - 12/5/2017 12:36:50 PM
lamin III zero. Liver function tests were all within normal limits. The bone marrow was impacted with imma ture cells and aspiration resulted in a dry tap. A trephine biopsy was performed and histopathology and touch imprints of the biopsy revealed mas sive infiltration with promyelocytes (fig. 1), some of which contained Auer rods. Treatment with daunomycin was started, plate let transfusions were administered. Nevertheless, severe thrombocytopenia persisted and massive gastrointestinal bleeding continued constantly throughout the hospitalization. Except for persis tent thrombocytopenia, repeated coagulation stud ies were all within normal limits, and there was no laboratory evidence of consumption coagulopathy. On the 15th day of hospitalization, the patient developed septicemia and died.
sification of acute leukemias: a survey of 1,400 cases. Blood Cells /; 7-15 (1975). 3 Gralnick, H. R.; Bargley, J., and Abrell, E.: Heparin treatment for the hemorrhagic dia thesis of acute promyelocytic leukemia. Am. J. Med. 52: 167-174 (1972). 4 Rachmilewitz, D.; Rachmilewitz, E. A.; Polliack, A., and Hcrshko, C.: Acute promyelocy tic leukemia: a report of five cases with a com ment on the diagnostic significance of serum vitamin B12 determination. Br. J. Haemat. 22: 87-92 (1972). 5 Pierce, J. I.; Borges, W. H.; Hcyn, R.; Wolff, J.
Engel hard/Yatziv/Rachmilewitz/Pol hack
A., and Gilbert, E. S.: Epidemiological factors and survival experience in 1,770 children with acute leukemia. Cancer 23: 1296-1304 (1969).
Received: February 8, 1979 Accepted: May 29, 1979 Prof. Aaron Pol hack, MD. Department of Hematology, Hadassah University Hospital, Jerusalem (Israel)
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