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|Year : 1992 | Volume
| Issue : 2 | Page : 96-7
Amegakaryocytic thrombocytopenic purpura.
K Harjai, M Shah, A Pant, P Kale, AV Pathare
Dr. J C Patel Hematology Department, Seth G S Medical College and KEM Hospital, Parel, Bombay.,
Dr. J C Patel Hematology Department, Seth G S Medical College and KEM Hospital, Parel, Bombay.
Keywords: Adult, Bone Marrow Examination, Case Report, Female, Human, Megakaryocytes, Platelet Count, Prednisone, administration &dosage,therapeutic use,Purpura, Thrombocytopenic, blood,diagnosis,drug therapy,
|How to cite this article:|
Harjai K, Shah M, Pant A, Kale P, Pathare A V. Amegakaryocytic thrombocytopenic purpura. J Postgrad Med 1992;38:96
Arnegakaryocytic thrombocytopenic purpura (ATP) is a hematological disorder characterised by severe thrombocytopenia, probably due to an immunologically induced absence of megakaryocPes in an otherwise normal appearing bone-marrow,. It may be a primary disorder itself or may be seen in aplastic anemia, preleukemia, and in systemic lupus erythematosus (SLE),,. It has also been reported in patients with Graves disease treated with radioiodine in the past in congenital rubella, dengue fever, nutritional B-12 deficiency ethanol abuse and certain congenital disorders like the TAR syndrome.
We are presenting here a case who probably had ATP and have reviewed the relevant literature.
SK, a 26-year-old housewife presented to us with a history of menorrhagia (of 20 days duration) since the last menstrual cycle. Her previous menstrual cycles were without any complaint. There was no history of fever, upper respiratory tract infection, diarrhoea, dysentery or recent exposure to any drugs or toxins.
Physical examination revealed numerous petechiae with some eechymotic patches on the trunk and the extremities. Investigations revealed Hb 8gm/dl, PCV 24%, retic count 1%, WBC 8.6 x 10/L with a normal differential and platelet count of 25 x 10/L. Bone-marrow aspiration showed a selective absence of megakaryocytes, with erythroid and myeloid precursors normal in number and morphology. A bone-marrow trephine biopsy revealed normal bone architecture with normal cellfularity. Erythropoiesis and granulopoiesis were normal but megakaryocytes were absent. Serological test for HIV, EBV, Hepatitis B virus, toxoplasma, syphillis, etc were negative. Her ANA, dsDNA and Coombs tests were also negative. However, anti-platelet antibodies were positive in a titer of 1:32. The patient did not show a remarkable improvement after starting prednisone in the dose of 1 mg/kg/day, However, her platelet count stabilised between 40 x 10 /L to 60 x 10/L and she became asymptomatic. Her excessive menstrual bleeding responded to hormonal therapy.
The pathogenesis of ATP may involve a defect in the early progenitor cell of the megakaryocytic lineage as seen by the deficient number of megakaryocytic colonies that can be grown from the bone-marrow aspirates of these patients, and by the absence of small platelet glycoprotein bearing mononuclear cells which are progenitors of megakaryocytes. Serum from such patients is reported to contain appropriately enhanced megalkaryocyte colony stimulating activity (CFU-M) in some, whereas it contains humoral inhibitor against CFU-M in others,. Chromium-tagged survival studies in these patients show a normal result, ruling out platelet destruction or sequestration.
While some patients remain clinically stable, others may progress to aplastic anemia or preleukemia in due course. In ATP due to SLE, steroids are known to affect a response, with an increase in the platelet count and T4:T8 ratio. This may indicate an immune-mediated etiology most likely a direct cell-to-cell interaction between patient's T-cells and megakaryocytic progenitor cells.
There have been sporadic reports of use of fresh frozen plasma FFP) and lithium carbonate in some of these patients,. The inducing effect of FFP on thrombopoietin production, probably due to the presence of a thrombopoietin activator or derepressor may be responsible for the beneficial effect. An occasional patient has been reported to benefit from lithium and cyclosporin. However, anabolic steroids, vincristine, methylprednisone and antilymphocytic globulin are hardly of any use. Thus, the only real hope today is with bone-marrow transplantation using a HLA-matched donor.
We wish to thank the Dean, Seth GS Medical College and King Edward Memorial Hospital, and Dr. GH Tilve, Prof and Head of the Dept. of Hematology, for allowing us to publish this case report.
| :: References|| |
Hoffman R, Bruno E, Elwell J, Mazur E, Gewirtz AM, Dekker P, Denes A, et al. Acquired amegakaryocytic thrombocytopenic purpura. A syndrome of diverse aetiologies. Blood 1982; 60:1173-1178. |
|2.||Rovira M, Feliu E, Florensa L. Acquired amegakaryocytic thrombocytopenic purpura associated with immunoglobulin deficiency. Acta Haematol 1991; 85:34-36. |
|3.||George JN, Aster RH. Thrombocytopenia due to diminished or defective platelet production. In: Williams WJ, Beutler E, Ersler AJ, Lichtman NA, Editors. Haematology 4th edition. New York: Hill Publishing Co; 1991; 1343-13451. |
|4.||Stoll DB, Blum S, Pasquale D. Thrombocytopenia with decreased megakaryocytes. Ann Int Med 1981; 94:170-175. |
|5.||Griner PH, Hoyer LW. Amegakaryocytic thrombocytopenia in systemic Jupus erythematosus. Arch Int Med 1970; 125:328-332. |
|6.||Nagasawa T, Sakurai T, Kashiwagi H, Abe T. Cellmediated amegakaryocytic thrombocytopenia associated with systemic lupus erythematosus. Blood 1986; 67:479-483. |
|7.||Sakurai T, Kono I, Kabashima T. Amegakarycytopenia associated with SLE successfully treated with high dose prednisone therapy. Jpn J Med 1984; 23:136-139. |
|8.||Sundstrom C, Kumberg D, Werner I. A case of thymorna in association with megakaryocytopenia. Acta Patho Microbiol Scand 1972; 80:487-490. |
|9.||Bayer WL, Sherman FE, Michaels RH, Szeto ILF, Lewis JH. Purpura in congenital and acquired rubella. N Engl J Med 1965; 273:1362-1365. |
|10.||Nelson ER, Bierman HR. Dengue fever. A thrombocytopenic disease? J Am Med Assoc 1964; 190:99-107. |
|11.||Ghos K, Sarode R, Verma N. Arnegakaryocytic thrombocytopenia of nutritional vitamin B12 deficiency. Trop Geogr Med 1988; 40:158-160. |
|12.||Gewirtz AM, Hoffinan R. Transitory hypomegakaryocytic thrombocytopenia. Aetiological association with ethanol abuse and implications regarding regulation of human megakaryopoiesis. Br J Haematol 1986; 62:333-344. |
|13.||Hedberg VA, Lipton JM. Thromobocytopenia with absent radii. A review of 100 cases. Am J Pediatr Haematol Oncol 1988; 10:51-64. |
|14.||Hill W, Landgraf R. Successful treatment of amegakaryocytic thrombocytopenic purpura with cyclosporine. N Engl J Med 1985; 312:1060-1061. |
|15.||Hirsh EH, Vogler WR, McDonald TP, Stein SF. Acquired hypomegakaryocytic thrombocytopenic purpura. Occurence in a patient with absent thrombopoietic stimulating factor. Arch Int Med 1980; 140:721-723.
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