A fatal case of transplantation-mediated alloimmune thrombocytopenia following liver transplantation

Figures & data

Figure 1. (a) Post-operative platelet count in a liver transplant recipient affected by transplantation mediated alloimmune thrombocytopenia (TMAT). (b) Timing of TMAT directed therapies. IV: intravenous; SC: subcutaneous; d: post-operative day; HAS: human albumin solution.

Figure 2. Thromboelastogram on day 10 post-transplantation. Thrombocytopenic picture. The reaction time (R) was normal, but clot formation time (K) was prolonged and maximum amplitude (MA) was decreased.

Figure 3. Tri-lineage extramedullary haematopoiesis in the donor liver. (a) Extramedullary haematopoiesis (original magnification ×50). Three megakaryocytes highlighted with arrows. (b) Lymphocytes around the portal tract (×10). (C) Erythroid precursors stained with glycophorin C (×50). (D) Lymphocytes present in the parenchyma and around the portal tract with CD3 (×10). (E) Myeloid precursors stained with myeloperoxidase (×50). (F) Three plasma cells in the portal tract highlighted with arrows (×50).

Table 1. Cases of transplantation mediated alloimmune thrombocytopenia (TMAT).

Study	Donor: (a) Cause of death (b) Preceding plt count (×10^9/l)	Donor: ITP history	Recipient: (a) Nadir plts (×10^9/l) (b) Nadir plts d0–3	Recipient: (a) Treatment (b) Outcome	Anti-platelet antibodies detected
Friend et al. [9]	(a) ICH (b) 5	ITP 26 years Prior ICH Prior Rx: steroids, splenectomy, cyclophosphamide	(a) 2 (b) <10 POD3	(a) Splenic rupture/resection d0, IVIg d4–8, Pl Ex d5–6 (b) ICH d7. Liver retransplant for rejection d11 Plts normal post re-transplant. Alive at 5 m	Donor: yes (GPIIb/IIIa) Recipient: yes (GPIIb/IIIa d5, negative by 5 m)
de la Torre et al. [15]	(a) ICH (b) 14	n/r	(a) 2 (b) 2 POD0	(a) IVIg.(d0,3) (b) Plts 43 d5 and normal at 24 m	Donor: no Recipient: PAIgG*
Diaz et al. [8]	(a) ICH (b) 19	ITP 21 years Prior ICH (×2) Prior Rx: steroids, splenectomy, IFN, cyclophosphamide, vincristine, danazol, ATG	(a) 2 (b) 6 POD1	(a) IVIg d2–6, 12, 39, 40. Rituximab d11, 15, 22, 29, 36 Vincristine d38, Pl Ex d9, 12. Splenectomy d13. Liver re-transplant d44 (b) Melena d2, intra-abdominal bleeding d7, hepatic subcapsular haematoma d43. Plts recovered post re-transplant. Septic death 2 m post discharge	Donor: n/r Recipient: PAIgG elevated d2
Pereboom et al. [16]	(a) ICH (b) 2	Fatal ICH 1 day after acute ITP presentation	(a) 1 (b) 6 POD3	(a) Steroids from d3, IVIg d3–4 (b) Plts improved from d8. Death from PVT and liver failure d13	Donor: yes Recipient: yes (specificity not stated)
Kaestner and Kutsogiainnis [10]	(a) ICH (b) 1	Fatal ICH as the acute ITP presentation	(a) 4 (b) 4 POD3	(a) Steroids from d3, IVIg d3, 5 (b) Bloody exudate from drains, sub-hepatic haematoma d5. Plts 58 d17 and 80–150 at 3 years	Donor: yes (GPIIb/IIIa) Recipient: yes (GPIIb/IIIa, negative by 1 m)
Current case	(a) ICH (b) 6	ITP 2 years Prior Rx: steroids, splenectomy, rituximab, romiplostim	(a) 2 (b) 2 POD2	(a) IVIg d3, 4, steroids d6–8, rituximab d7, romiplostim d8, 17, plasmapheresis d10, vincristine d11, SAE d14, rFVII d17 (b) Melena from d3, haemoptysis from d5. Death from PH and MOF d18	Donor: yes (GPIb/IX) Recipient: yes (GPIb/IX)

Note: ICH: intracranial haemorrhage; ITP: immune thrombocytopenia; GP: glycoprotein; d: post-operative day; IVIg: intravenous immunoglobulin; Plt: platelet; Rx: treatment; m: month; n/r: not reported; IFN: interferon; Pl Ex: plasma exchange; ATG: anti-thymocyte globulin; PH: pulmonary haemorrhage; MOF: multiorgan failure; PVT: portal vein thrombosis; PAIgG: platelet-associated IgG.

*Detected day 2 but negative 2 weeks after transplantation.

Friend PJ, McCarthy LJ, Filo RS, Transmission of idiopathic (autoimmune) thrombocytopenic purpura by liver transplantation. N Engl J Med. 1990 Sep 20;323(12):807–811. doi: 10.1056/NEJM199009203231207

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Diaz GC, Prowda J, Lo IJ, et al. Transplantation-mediated alloimmune thrombocytopenia: guidelines for utilization of thrombocytopenic donors. Liver Transpl. 2008 Dec;14(12):1803–1809. doi: 10.1002/lt.21539

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Pereboom IT, de Boer MT, Haagsma EB, et al. Transmission of idiopathic thrombocytopenic purpura during orthotopic liver transplantation. Transpl Int. 2010 Feb;23(2):236–238. doi: 10.1111/j.1432-2277.2009.00936.x

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Kaestner F, Kutsogiannis DJ. Successful kidney and liver transplantation from a donor with immune thrombocytopenia. Transplant Proc. 2013 Sep;45(7):2838–2840. doi: 10.1016/j.transproceed.2013.02.136

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