Acquired thrombotic thrombocytopenic purpura (aTTP) is a serious disorder with arteriolar and capillary thrombosis for which the treatment usually requires plasma exchange with plasma as the replacement fluid. Management of patients who do not accept blood products is a serious challenge. We present the case of a Jehovah's Witness patient who achieved clinical response after treatment with plasma exchange using human albumin solution as the replacing fluid, high dose corticosteroids, and rituximab. The patient also received ADAMTS13 containing plasma cryoprecipitate and von Willebrand factor VIII concentrates. She had an exacerbation of her TTP in less than 3 weeks. She was treated with further plasma exchange with human albumin solution as the replacement fluid, high dose steroids, and rituximab. Bortezomib and N-acetylcysteine were added. The patient eventually improved clinically and achieved remission that is ongoing for more than 7 months. A review of the literature shows that all five previously reported cases of aTTP in Jehovah's Witnesses survived although none received plasma. Two were not even treated with plasma exchange. The experience of this case and those in the literature demonstrates that remission of aTTP may be achieved without using plasma or plasma exchange.

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Introduction: Pseudo-thrombocytopenia (PTCP) is a benign artifact which generates anxiety in the patients and physicians resulting in unnecessary investigations. We report a case with profound artefactual thrombocytopenia. Case: A 31-years-old female in 38th week of gestation was referred to hematology clinic for evaluation of a platelet count of 48x109/L. A month ago platelet count was 102x109/L. She denied any symptoms. Examination was normal with no ecchymosis or petechiae. Laboratory work showed a white cell count of 11.2x109/L, hemoglobin 10.3g/L, platelet count 8x109/L and no hemolysis. Peripheral blood smear showed numerous platelet clumps (Figure 1). Estimated manual platelet count was 200x109/L. To our surprise clumps and low machine count were seen in samples drawn in sodium citrate and heparin tubes as well. Discussion: Ethylenediaminetetra-acetic acid (EDTA) dependent PTCP is a rare phenomenon with incidence of 0.09%-0.21% in general population (1). It is present in healthy subjects (2), severely ill patients with sepsis (3), autoimmune, neoplastic and liver diseases (4). PTCP may persist for 15-20 years without any clinical manifestations (5). Platelet aggregation in PTCP is due to anti-platelet antibody mediated in-vitro activation via GPIIb receptors (6). Antibodies may be IgG, IgA or IgM (5). It may be seen in samples drawn in citrate (5) heparin (7) and sodium-oxalate (8). One should suspect PTCP when there is fall in platelet count (usually <100x109/L), time-dependent spurious elevation of white cells(9), a normal mean platelet volume (4) or microscopic detection of platelet aggregates (1) in a patient without clinical manifestations. There is a rare report that addition of amikacin could inhibit and dissociate pseudo platelet aggregation in multianticoagulant-dependent pseudo-thrombocytopenia and EDTA-induced pseudo-thrombocytopenia (10). Early identifications is extremely essential when therapeutic decision making hinges on platelet count viz. management of acute myocardial infarction (11).Use of samples at 37°C (2), ammonium oxalate (5, 7), addition of amikacin and peripheral smear review may help in complicated cases. References: 1. Yoneyama A, Nakahara K. [EDTA-dependent pseudothrombocytopenia--differentiation from true thrombocytopenia]. Nihon Rinsho. 2003;61(4):569-74. 2. Lippi G, Plebani M. EDTA-dependent pseudothrombocytopenia: further insights and recommendations for prevention of a clinically threatening artifact. Clin Chem Lab Med. 2012;50(8):1281-5. 3. Mori M, Kudo H, Yoshitake S, Ito K, Shinguu C, Noguchi T. Transient EDTA-dependent pseudothrombocytopenia in a patient with sepsis. Intensive Care Med. 2000;26(2):218-20. 4. Berkman N, Michaeli Y, Or R, Eldor A. EDTA-dependent pseudothrombocytopenia: a clinical study of 18 patients and a review of the literature. Am J Hematol. 1991;36(3):195-201. 5. Bizzaro N. EDTA-dependent pseudothrombocytopenia: a clinical and epidemiological study of 112 cases, with 10-year follow-up. Am J Hematol. 1995;50(2):103-9. 6. Fiorin F, Steffan A, Pradella P, Bizzaro N, Potenza R, De Angelis V. IgG platelet antibodies in EDTA-dependent pseudothrombocytopenia bind to platelet membrane glycoprotein IIb. Am J Clin Pathol. 1998;110(2):178-83. 7. Zandecki M, Genevieve F, Gerard J, Godon A. Spurious counts and spurious results on haematology analysers: a review. Part I: platelets. International Journal of Laboratory Hematology. 2007;29(1):4-20. 8. Schrezenmeier H, Muller H, Gunsilius E, Heimpel H, Seifried E. Anticoagulant-induced pseudothrombocytopenia and pseudoleucocytosis. Thromb Haemost. 1995;73(3):506-13. 9. Xiao Y, Xu Y. Concomitant spuriously elevated white blood cell count, a previously underestimated phenomenon in EDTA-dependent pseudothrombocytopenia. Platelets. 2015;26(7):627-31. 10. Zhou X, Wu X, Deng W, Li J, Luo W. Amikacin can be added to blood to reduce the fall in platelet count. Am J Clin Pathol. 2011 Oct;136(4):646-52. 11. Kocum TH, Katircibasi TM, Sezgin AT, Atalay H. An unusual cause of mismanagement in an acute myocardial infarction case: pseudothrombocytopenia. Am J Emerg Med. 2008;26(6):740 e1-2. Figure 1 Platelet Clumps- Magnification 60X Oil Figure 1. Platelet Clumps- Magnification 60X Oil Disclosures No relevant conflicts of interest to declare.