Successful treatment of acquired amegakaryocytic thrombocytopenic purpura refractory to corticosteroids and intravenous immunoglobulin with antithymocyte globulin and cyclosporin

Niparuck, Pimjai; Atichartakarn, Vichai; Chuncharunee, Suporn

doi:10.1007/s12185-008-0125-0

Cited by 15 publications

(16 citation statements)

References 12 publications

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“…Based on the proposed humoral immunity mechanism, various immunosuppressive therapeutic approaches have been utilised in patients with AAT. Several case series have reported successful treatment with cyclosporine as a mono-therapy or in combination with other immunosuppressive agents [7]. Our patient was treated with cyclosporine and steroids with platelet transfusion.…”

Section: Discussionmentioning

confidence: 82%

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Acquired Amegakaryocytic Thrombocytopenia in a Child: A Very Rare Case

Mishra

et al. 2016

HTIJ

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Section: Discussionmentioning

confidence: 82%

“…Our patient was treated with cyclosporine and steroids with platelet transfusion. In few published literature patients were treated with ATG, Danazol, myeloablative chemotherapy, cyclophosphamide, IVIG, blood and marrow stem cell transplantation [7][8][9][10]. So, far cyclosporine has shown to be the best therapeutic intervention.…”

Section: Discussionmentioning

confidence: 99%

Acquired Amegakaryocytic Thrombocytopenia in a Child: A Very Rare Case

Mishra

et al. 2016

HTIJ

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“…The exact prevalence is unknown and the available literature comprises case reports and small case series. It is possible that the incidence rate is higher than what is reported and that many of the cases are underdiagnosed or misdiagnosed as immune thrombocytopenia [10]. The clinical course of this rare disease seems to be variable.…”

Section: Discussionmentioning

confidence: 91%

“…The administration of corticosteroids, IVIG, cyclophosphamide, vincristine, androgens, and mycophenolate mofetil are transiently effective in occasional patients with AATP [1,15,16,20]; however, the administration of cyclosporine alone or in combination with ATG was shown to be very effective in the treatment of AATP [10,15,17,18].…”

Section: Discussionmentioning

confidence: 99%

“…APMA can be either idiopathic or caused by a variety of conditions, such as acquired clonal cytogenetic abnormalities, drug sensitivity, toxin exposure, infectious diseases such as viral infection [1,2], immune diseases such as lupus erythematosus [3], systemic sclerosis [4], eosinophilic fasciitis [5], and malignancy [6,7]. Patients with acquired amegakaryocytic thrombocytopenia may have additional hematological abnormalities such as macrocytosis or dyserythropoiesis, abnormalities which may indicate potential future progression to aplastic anemia or myelodysplasia [8][9][10].…”

Section: Introductionmentioning

confidence: 99%

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Acquired pure megakaryocytic aplasia successfully treated with cyclosporine

Omri¹,

Ibrahim²,

Taha³

et al. 2010

Turk J Hematol

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Acquired pure megakaryocytic aplasia is a rare hematological disorder characterized by thrombocytopenia with absent or markedly reduced megakaryocytes in the bone marrow. We report a case of a 25-year-old male diagnosed as acquired pure megakaryocytic aplasia. Treatment with prednisone and intravenous immunoglobulin failed, but he was successfully treated with cyclosporine, with complete remission after 90 days and normal platelet count maintained thereafter. (Turk J Hematol 2010; 27: 289-93) Key words: Acquired amegakaryocytic thrombocytopenia, steroids, cyclosporine

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Effective use of rituximab for acquired amegakaryocytic thrombocytopenia

Deeren

Dorpe

2010

American J Hematol

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Between 1981 and 2010, we have treated 40 patients requiring chronic transfusion with periodic manual erythrocyte exchange and autologous plasma rescue (MEEX). Here, we present retrospective, long-term (median 22, range 14-29 years) follow-up data for a subset of seven patients with sickle cell syndromes who did not respond to hydroxyurea (HU). Patient characteristics are listed in Supporting Information, Tables I and II. MEEX was accomplished by using a single venous access and infusing 500 ml of isotonic solution (for adults) and removing 500 ml blood (range 400-600 ml according to the patient's weight and the physician's discretion). The rescue process involves centrifugation of the collected blood product and reinfusion of autologous plasma. This is followed by another 500 ml phlebotomy and the infusion of 2-3 units of packed Rh-matched, leuko-filtered, plasma-depleted red cells, with the aim of lowering HbS levels to around 40%. For pediatric patients, the bleeding volume is smaller (5-10 cc/Kg) and the volumes of infused saline and packed Rhmatched, leuko-filtered, plasma-depleted red cells are equal to the phlebotomy volumes, without plasma rescue.The interval between each MEEX procedure for these seven patients ranged from 45 to 90 days to maintain at all times HbS concentrations below 60-70% and the median number of procedures was 133 (range 85-204). None of the seven patients experienced acute complications of sickle cell disease (SCD), such as acute chest syndrome, splenic sequestration, stroke, bone necrosis, or priapism, or long-term complications such as renal failure, cerebrovascular or retinal damage, or pseudoxanthoma-like manifestations. None of the seven patients experienced alloimmunization and all but one patient had normal liver iron concentrations [assessed by superconducting quantum interference device, magnetic iron detector, magnetic resonance imaging (MRI), or biopsy]. This patient, who started the program at the age of 26 years with iron overload due to previous transfusions, required regular iron chelation therapy. Cardiac function tests indicated that all subjects had a left ventricular ejection fraction of >60% and cardiac T 2 * > 20 mms (measured by MRI). There was no evidence of pulmonary arterial hypertension on echocardiography.During the observation period, five patients required hospitalization (four with acute cholecystitis and one due to venous occlusive crisis). Only one patient requires chronic analgesia (to relieve pain caused by pre-existing femur head necrosis).Automated EEX (AEEX) ensures lower postprocedural HbS levels (30% HbS vs. 40% with our MEEX protocol) because it makes use of two distal ports to increase the volume of red cells removed. However, the larger number of transfused units in AEEX exposes the patient to a higher transfusional risk. In addition, the automated procedure is significantly more expensive than the manual one, as indicated in Table I.Previous studies have indicated that EEX is a safe and efficacious alternative to both HU and simple transfu...

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Successful treatment of acquired amegakaryocytic thrombocytopenic purpura refractory to corticosteroids and intravenous immunoglobulin with antithymocyte globulin and cyclosporin

Cited by 15 publications

References 12 publications

Acquired Amegakaryocytic Thrombocytopenia in a Child: A Very Rare Case

Acquired Amegakaryocytic Thrombocytopenia in a Child: A Very Rare Case

Acquired pure megakaryocytic aplasia successfully treated with cyclosporine

Effective use of rituximab for acquired amegakaryocytic thrombocytopenia

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