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Arteropathies, Microcirculation and Vasculitis
Published in Mary N. Sheppard, Practical Cardiovascular Pathology, 2022
TTP is a thrombotic microangiopathy with an incidence of 3.7–11 per million cases. TTP is characterized by thrombocytopaenia, macroangiopathic haemolytic anaemia, fever, neurological and renal impairment. TTP can be primary or secondary. Secondary TTP is associated with cancer, drugs, pregnancy and collagen vascular diseases. TTP needs to be differentiated from DIC, haemolytic uremic syndrome (HUS), HELLP syndrome in pregnancy, drug manifestations and TMA. Females with obesity and Afro-Caribbean ancestry are at increased risk. Cardiac involvement in patients with TTP is well established. TTP shares histological features with DIC but is clinically distinct. The diagnosis of TTP is established on histological findings and identification of thrombocytopaenia with schistocytes on premortem blood smear. Underlying conditions for TTP can be infection, autoimmune disease, sickle cell disease, HIV infection, cocaine abuse and idiopathic. In the myocardium, widespread presence of intracapillary platelet-rich thrombi, in contrast to DIC, which is characterized by predominantly fibrin thrombi. Antibodies to platelet glycoprotein IIIa (CD61) and fibrin II is helpful in diagnosing TTP and distinguishing it from DIC.29
Congenital and acquired disorders of coagulation
Published in Jennifer Duguid, Lawrence Tim Goodnough, Michael J. Desmond, Transfusion Medicine in Practice, 2020
Jeanne M Lusher, Roshni Kulkarni
Treatment of HUS in children consists of supportive care. Adults with TTP are often treated with transfusion of FFP or plasma exchange using FFP, or cryoprecipitate-poor plasma (to avoid giving vWF). The survival with plasma exchange is 80%. Other treatment modalities with variable success have included corticosteroids, splenectomy, vincristine, antiplatelet agents such as aspirin, and dipyridamole.
Immunohematology
Published in Gabriel Virella, Medical Immunology, 2019
Gabriel Virella, Armand Glassman
The trigger for the formation of ADAMTS13 antibody generation is usually unknown although there have been associations with some medications, pregnancy, lupus erythematosus and other autoimmune diseases, some neoplasias and human immunodeficiency disease. The very rare form of inherited TTP is associated with autosomal recessive inheritance of the absence of ADAMTS13. The diagnosis is most usually based on clinical and laboratory parameters but can be confirmed by determining ADAMTS13 levels (less than 5% of normal values are diagnostic).
An update on the pathogenesis and diagnosis of thrombotic thrombocytopenic purpura
Published in Expert Review of Hematology, 2023
Inés Gómez-Seguí, Cristina Pascual Izquierdo, María Eva Mingot Castellano, Javier de la Rubia Comos
Based on clinical practice, the complementary tests recommended in the diagnosis and follow-up of patients with TTP are described in detail in Table 3. Briefly, it is worth mentioning that in the case of ischemia in an atypical location (pancreatitis, intestinal ischemia, etc.) it is recommended to request targeted complementary tests such as amylase and lipase levels and imaging tests. On the other hand, although venous thromboembolic events are infrequent and are generally related to the use of catheters, in the case of clinical suspicion, it is recommended to request the appropriate imaging tests since D-dimer is not useful for screening in these patients [112]. After remission, follow-up is necessary, mainly to avoid recurrences and relapses. The frequency of these visits has not been established, but could be monthly for 3 months after remission, quarterly for 9 months every 6 months for at least 2 years, and then yearly [118,123].
Inherited ADAMTS13 mutations associated with Thrombotic Thrombocytopenic Purpura: a short review and update
Published in Platelets, 2023
Zoe Markham-Lee, Neil V. Morgan, Jonas Emsley
Thrombotic Thrombocytopenic Purpura (TTP) is a rare blood disorder, associated with excessive thrombus formation in the microvasculature. This leads to a variety of symptoms ranging dramatically in their severity. Mild clinical manifestations include headaches and lethargy, through to potentially fatal manifestations of transient ischemic attack, stroke, myocardial damage or renal failure. In total, 75% of patients were female and 25% male according to the regional UK TTP registry (2008) [3]. Diagnosis of TTP relies foremost on ADAMTS13 activity (threshold of <10%), due to lack of uniformity in symptoms and respective severity across patients. The congenital form of disease (cTTP) is associated with biallelic mutations (autosomal recessive inheritance) in the ADAMTS13 gene, whilst the immune-mediated form (iTTP) involves autoantibody targeting of the ADAMTS13 protein; the acquired form is more prevalent (95% of cases). cTTP is confirmed by direct sequence analysis of the ADAMTS13 gene; defects may be homozygous or compound heterozygous [3].
Successful use of a second course of caplacizumab in relapsed thrombotic thrombocytopenic purpura
Published in Platelets, 2022
A 25-year-old woman with no prior history of TTP presented to the emergency department with abdominal pain, bilateral lower extremity bruising, and nausea for the past week. Past medical history included obesity, type 2 diabetes mellitus, and hypothyroidism. Initial labs revealed a platelet count of 5 × 109/L, hemoglobin of 6.6 g/dL, haptoglobin of <10 mg/dL, indirect bilirubin of 5 mg/dL, and lactate dehydrogenase 1,418 units/L. She was also noted to have occasional schistocytes on the peripheral blood smear suggestive of microangiopathic hemolytic anemia. ADAMTS13 activity later resulted as <5% confirming the diagnosis of TTP. Daily PE and corticosteroids (methylprednisolone 10 mg/kg/day for 3 days followed by 1 mg/kg/day) were initiated on day 1 with weekly rituximab 375 mg/m2 added on day 4. On day 10, she had a right frontal stroke secondary to TTP and platelets had not improved, which led to the decision to start caplacizumab. Caplacizumab was obtained and initiated on day 12 with 11 mg intravenously prior to PE on day 1 followed by 11 mg subcutaneously after PE and continued daily until 30 days after the last PE session. Following initiation of caplacizumab, platelets trended up to 203 × 109/L by day 18 and she was able to be discharged on day 25 with platelets of 553 × 109/L. Upon completion of the course of caplacizumab on day 46, platelets were 292 × 109/L.