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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.
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