Acquired amegakaryocytic thrombocytopenia (AAT), a rare entity characterized by severe thrombocytopenia and the absence of megakaryocytes in the bone marrow, may mimic or precede the diagnosis of aplastic anemia (AA). Here, we describe a patient who presented with apparent Epstein-Barr virus (EBV)-associated immune thrombocytopenia resistant to several lines of therapies, which was in fact a form of AAT with some features of AA. He eventually responded to therapy with eltrombopag, cyclosporine A (CSA), and antithymocyte globulin (ATG) and recovered completely. EBV infection is known to cause a variety of benign and malignant hematologic disorders, including bone marrow failure. However, to the best of our knowledge, this is the first case report of EBV-associated AAT. Treatment options for AAT are still not well defined, and even response to eltrombopag together with CSA and ATG does not always imply successful therapy. The natural history of EBV infection may well be sufficient to explain unexpected eventual recovery.

Tristano AG: Acquired amegakaryocytic thrombocytopenic purpura: review of a not very well-defined disorder. Eur J Intern Med 2005;16:477-481.
Agarwal N, Spahr JE, Werner TL, Newton DL, Rodgers GM: Acquired amegakaryocytic thrombocytopenic purpura. Am J Hematol 2006;81:132-135.
Erkurt MA, Kaya E, Baran M, Yitmen E, Şenel S, Kuku İ, Aydoğdu İ: Rapid progression of acquired amegakaryocytic thrombocytopenia to myelodysplastic syndrome: case report. Turk J Haematol 2005;22:205-208.
Lonial S, Bilodeau PA, Langston AA, Lewis C, Mossavi-Sai S, Holden JT, Waller EK: Acquired amegakaryocytic thrombocytopenia treated with allogeneic BMT: a case report and review of the literature. Bone Marrow Transplant 1999;24:1337-1341.
Cela I, Miller I, Katz R, Rizman A, Shammo J: Successful treatment of amegakaryocytic thrombocytopenia with eltrombopag in a patient with systemic lupus erythematosus (SLE). Clin Adv Hematol Oncol 2010;9:806-809.
Ernestho-ghoud IM, Rahamefy O, Ranaivo IM, Andrianarison M, Ramarozatovo LS, Rabenja FR: Acquired amegakaryocytic thrombocytopenic purpura: think of systemic lupus erythematosus (in French). Pan Afr Med J 2015;20:1-4.
Colovic M, Pavlovic S, Kraguljac N, Colovic N, Jankovic G, Sefer D, Tosic N: Acquired amegakaryocytic thrombocytopenia associated with proliferation of γ/δ TCR+ T-lymphocytes and a BCR-ABL (p210) fusion transcript. Eur J Haematol 2004;73:372-375.
Lai DW, Loughran TP Jr, Maciejewski JP, Sasu S, Song SX, Epling-Burnette PK, Paquette RL: Acquired amegakaryocytic thrombocytopenia and pure red cell aplasia associated with an occult large granular lymphocyte leukemia. Leuk Res 2008;32:823-827.
Shichishima T, Ikeda K, Takahashi N, Kameoka J, Tajima K, Murai K, Tamai Y, Shichishima-Nakamura A, Akutsu K, Noji H, Okamoto M, Kimura H, Harigae H, Oyamada T, Kamesaki T, Takeishi Y, Sawada K: Low concentration of serum haptoglobin has impact on understanding complex pathophysiology in patients with acquired bone marrow failure syndromes. Int J Hematol 2010;91:602-610.
Onuki T, Kiyoki Y, Ueda S, Yamaoka M, Shimizu S, Inagaki M: Invasive thymoma with pure red cell aplasia and amegakaryocytic thrombocytopenia. Hematol Rep 2016;8:61-63.
Gay CM, William WN, Wang SA, Oo TH: Thymoma complicated by acquired amegakaryocytic thrombocytopenia and pure red cell aplasia. J Natl Compr Canc Netw 2014;12:1505-1509.
Okano M: Haematological associations of Epstein-Barr virus infection. Baillieres Best Pract Res Clin Haematol 2000;13:199-214.
Pipp ML, Means ND, Sixbey JW, Morris KL, Gue CL, Baddour LM: Acute Epstein-Barr virus infection complicated by severe thrombocytopenia. Clin Infect Dis 1997;25:1237-1239.
Baranski B, Armstrong G, Truman JT, Quinnan GV Jr, Straus SE, Young NS: Epstein-Barr virus in the bone marrow of patients with aplastic anemia. Ann Intern Med 1988;109:695-704.
Cai Q, Chen K, Young KH: Epstein-Barr virus-positive T/NK-cell lymphoproliferative disorders. Exp Mol Med 2015;47:e133.
Brown GE, Babiker HM, Cantu CL, Yeager AM, Krishnadasan R: “Almost bleeding to death”: the conundrum of acquired amegakaryocytic thrombocytopenia. Case Rep Hematol 2014;2014:806541.
Jain S, Kapoor S, Singh A, Ravi RNM, Jain S: Good response to cyclosporin in a child with acquired thrombocytopenic purpura. Indian J Pediatr 2012;79:1236-1237.
Shigekiyo T, Sekimoto E, Shibata H, Ozaki S, Fujinaga H, Hirose T: Treatment of acquired amegakaryocytic thrombocytopenic purpura with romiplostim. Platelets 2015;26:504-506.
Ueda K, Matsubara A, Kizuki N, Sato Y, Oka Y, Hosaka T: Successful treatment of acquired pure red cell aplasia and acquired amegakaryocytic thrombocytopenia with anti-thymocyte globulin. Am J Hematol 2001;66:154.
Chang H, Tang TC: Successful treatment of amegakaryocytic thrombocytopenia with azathioprine. Acta Haematol 2011;126:135-137.
Defrien D, Van Dorpe J: Effective use of rituximab for acquired amegakaryocytic thrombocytopenia. Am J Hematol 2010;85:977-978.
Hoffman R: Acquired pure amegakaryocytic thrombocytopenic purpura. Semin Hematol 1991;28:303-312.
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