Post-Partum Thrombotic Thrombocytopenic Purpura (TTP) in a Patient with known Idiopathic (Immune) Thrombocytopenic Purpura: a case report and review of the literature

Abstract

BackgroundIncidences of immune thrombocytopenic purpura occur in 1 in every 1000–10,000 pregnancies accounting for 3% of all thrombocytopenic pregnancies. A pre-existing immune thrombocytopenic purpura is known to be a risk factor for developing thrombocytopenia during pregnancy. We present here the treatment regime and management of a patient with known immune thrombocytopenic purpura who developed postpartum thrombotic thrombocytopenia with atypical response to traditional therapy.Pregnant women are more vulnerable to immune thrombocytopenic purpura or thrombotic thrombocytopenia. Pregnancy or postpartum thrombotic thrombocytopenia accounts for 10–25% of all thrombotic thrombocytopenia.Case presentationThis case report deals with the treatment regime and management of a patient with known immune thrombocytopenic purpura who developed postpartum thrombotic thrombocytopenia. A 30-year-old Middle Eastern woman, with a prior diagnosis of chronic immune thrombocytopenic purpura had remained off-the-treatment for many years. After primary unexplained infertility for 8 years, for which she underwent six failed trials of in vitro fertilization, she delivered a healthy baby through caesarean section. Two days post-surgery, she had persistent thrombocytopenia, ecchymoses, bruises, and hemolysis. Her blood film revealed leukoerythroblastic anemia. Her blood tests also revealed a very low level of haptoglobin, and low level of ADAMTS13. A diagnosis of thrombotic thrombocytopenia was suspected. Plasma exchange therapy was started with partial response. We showed that rituximab in conjunction with mycophenolate mofetil following plasma exchange therapy was effective in controlling the low platelet count in our patient.ConclusionsRituximab in conjunction with mycophenolate mofetil following plasma exchange therapy was effective in controlling the low platelet count in our patient. Only two doses of rituximab were sufficient to normalize our patient. We present here a case of safe and effective use of rituximab in pregnancy-induced thrombotic thrombocytopenia.

Thrombotic thrombocytopenic purpura with Graves’ disease during pregnancy

“…In addition, preexisting ITP is a known risk factor for developing TTP during pregnancy. 14 Our patient had recurrent thrombocytopenia, and her ADAMTS13 level met the diagnostic criteria for TTP. However, the TTP subtype (congenital vs acquired) remains unknown since anti-ADAMTS13 IgG levels and genetic analysis were not performed.…”

“…Extremely low platelet levels, as seen in this patient, are known to cause intramyocardial hemorrhage, 16 and myocardial ischemia due to platelet thrombi was contributory to this death. Also, pregnancy is clearly a trigger for the development of ITP and TTP, 12,14,15,17 warranting a high index of suspicion in pregnant patients who develop thrombocytopenia.…”

Successful Rituximab Treatment in Thrombotic Thrombocytopenic Purpura Patients Complicated by Other Autoimmune Disorders: Two Case Reports

“…There are few reports describing TTP complicated by AIH or ITP (3,4). It remains unclear whether there are differences in the clinical courses and outcomes between TTP patients with or without other autoimmune disorders.…”

Refractory Severe Thrombocytopenia during Pregnancy: How to Manage

“…3,6 This condition may be asymptomatic, diagnosed from routine laboratory tests, or may manifest more rarely as severe cases with petechiae, hemorrhages and hematomas. 7 Treatment is indicated for platelet counts below 30 Â 10 9 /L, with steroids and IgG as first line treatment, 8,9 and alternatively, thrombopoietin receptors agonists, 10 rituximab, 11 or, eventually, splenectomy (occasionally performed and, if necessary, in the 2 nd trimester of gestation). 12 Since there is no treatment with guaranteed effectiveness, many studies have shown that the clinical management of pregnancy, delivery, and puerperium in patients with thrombocytopenia needs a close cooperation of experienced hematologists, obstetricians, anesthesiologists and neonatologist.…”