Thrombotic thrombocytopenic purpura in a patient with hemoglobin E disease-the importance of timely examination of a blood film

2012 ◽  
Vol 87 (10) ◽  
pp. 996-996 ◽  
Author(s):  
Biswadip Hazarika ◽  
Barbara J. Bain
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Blood Film ◽  
Thrombocytopenic Purpura ◽  
Hemoglobin E ◽  
Hemoglobin E Disease

scholarly journals Occult Disseminated Metastatic Breast Carcinoma Presenting as Acquired Thrombotic Thrombocytopenic Purpura

2021 ◽  
pp. 1814-1820
Author(s):  
Siew Lian Chong ◽  
Asral Wirda Ahmad Asnawi ◽  
Roszymah Hamzah ◽  
Pek Kuen Liew ◽  
Tee Chuan Ong ◽  
...  
Keyword(s):  
Breast Carcinoma ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Hemolytic Anemia ◽  
Metastatic Breast ◽  
Metastatic Carcinoma ◽  
Blood Film ◽  
Thrombocytopenic Purpura ◽  
Metastatic Breast Carcinoma ◽  
Threatening Condition ◽  
Life Threatening

Cancer-related microangiopathic hemolytic anemia (MAHA) is a rare and life-threatening condition. We present a patient who had been treated for invasive lobular breast carcinoma in clinical remission with fever and hemolytic anemia. The peripheral blood film showed MAHA and thrombocytopenia, and a functional deficiency of ADAMTS13 activity of 23% consistent with acquired thrombotic thrombocytopenic purpura. Bone marrow aspirate and trephine biopsy confirmed metastatic carcinoma. Further evaluation revealed the involvement of multiple bone sites without recurrence of the primary tumor. The patient received a daily plasma exchange with cryosupernatant and was pulsed with corticosteroids. MAHA related to breast cancer appears to be a rare occurrence.

HIV-Associated Thrombotic Thrombocytopenic Purpura

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4677-4677 ◽  
Author(s):  
Jordana Boro ◽  
Katerina Pavenski ◽  
Kimberley L.S. Ambler ◽  
Heather A Leitch ◽  
Lisa K Hicks
Keyword(s):  
Hiv Infection ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Case Series ◽  
Blood Film ◽  
Vancouver General Hospital ◽  
Neurological Deficits ◽  
Limited Information ◽  
Thrombocytopenic Purpura ◽  
Cd4 Counts ◽  
Small Series

Abstract Abstract 4677 Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening form of microangiopathic hemolysis that can be associated with HIV infection, and has previously been reported to be associated with low CD4 counts. The existing literature on HIV-TTP is very small and largely made up of small case series generated through databases of patients with HIV. As a result, reports have tended to focus on HIV parameters with limited information available regarding the presentation, management and outcome of the TTP itself. We conducted a retrospective review of TTP cases referred to the pheresis units St. Michael’s Hospital and Vancouver General Hospital in Vancouver between July 1993 and May 2011. Ten cases of HIV associated TTP were identified (8 male; 2 female). Median age at presentation was 38 years. One patient had been previously diagnosed and treated for TTP at a different institution. Average duration of HIV infection prior to TTP diagnosis was 5 years (range 0 to 11). Median CD4 count at TTP diagnosis was 79 × 106/ml (range 2 to 326). Median platelet count at presentation was 14 × 106/ml (range 5 to 233), median haemoglobin was 74 g/L (range 61 to 133), all patients had an LDH > 2x the upper limit of normal, and all for whom data was available (8/10) had fragmentation on blood film. Creatinine was elevated in 9 of 10 patients. ADAMTS13 was assessed in 3 of 10 patients and was deficient in one. Five of 10 patients had fever. At presentation, 6 of 10 patients had neurological symptoms (most commonly seizures and/or confusion), but none suffered permanent neurological deficits. All patients were treated with plasmapheresis and received a median of 16 exchanges (2-56). Four patients received concurrent steroids, two patients received IVIG, and one patient received pheresis, steroids IVIG, vincristine and rituximab. Eight of 10 patients achieved complete remissions, one patient achieved a partial response, and one died on treatment. Four of the responding patients subsequently relapsed (0.6 to 13.8 months after the initial episode of TTP); two achieved second remissions, and two died on treatment. In conclusion, our series of HIV associated-TTP confirms previous reports that HIV-TTP tends to occur in patients with CD4 counts less than 200. Complete remissions can be achieved with standard management. However, based on our small series, relapses may be more common and mortality greater than in the general TTP population. Disclosures: Leitch: Novartis Pharmaceuticals: Honoraria, Membership on an entity’s Board of Directors or advisory committees.

Successful Pregnancy Outcome in 5 Patients with Thrombotic Thrombocytopenic Purpura.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3016-3016
Author(s):  
Marie Scully ◽  
Richard Starke ◽  
Richard Lee ◽  
Ian Mackie ◽  
Samuel J. Machin ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Pregnancy Outcome ◽  
Post Partum ◽  
Blood Film ◽  
Activity Levels ◽  
Laboratory Monitoring ◽  
Igg Antibodies ◽  
Successful Pregnancy ◽  
Thrombocytopenic Purpura ◽  
History Of

Abstract Pregnancy is an initiating event for acute thrombotic thrombocytopenic purpura (TTP). Patients with a history of TTP have a high risk of relapse during pregnancy with associated maternal and fetal morbidity/mortality. We report 5 TTP patients who underwent serial clinical and laboratory monitoring (haemoglobin, platelets, blood film, reticulocytes, lactate dehydrogenase (LDH), ADAMTS-13 activity and IgG antibodies) and treatment within a specialist obstetric/haematology clinic, resulting in successful pregnancy outcomes. Patients: Cases 1 and 2 presented with TTP in their first pregnancy and had second trimester fetal losses. Case 3 had four TTP relapses and soon after achievement of clinical remission became pregnant. Case 4 had a single acute episode of TTP 6 years prior to pregnancy and normal ADAMTS 13 activity at the onset of pregnancy. Case 5 presented with acute TTP and a left sided stroke at 16 weeks gestation. ADAMTS-13 activity was <5% at onset of pregnancy in cases 1–3 and at presentation of TTP during pregnancy in case 5. Only case 3 had IgG antibodies to ADAMTS13. Cases 1–3 received regular plasma exchange (PEX) every 2 weeks initially, starting during the first trimester, with ADAMTS 13 activity levels maintained >15% during pregnancy (range 15–78%, normal range 66–126%) by collagen binding assay. Case 4 had normal laboratory parameters, including serial ADAMTS 13 activity levels, throughout pregnancy, requiring no specific therapy for TTP. Case 5 received intensive PEX (84 procedures) and pulsed methylprednisolone during pregnancy. Rituximab (375mg/m2, weekly for 4 weeks) was given at 28 weeks gestation with no observed toxicity except asymptomatic transient neonatal neutropenia. All 5 cases continued low dose aspirin (75 mg daily) throughout pregnancy. Case 1 and 2 received prophylactic low molecular weight heparin (LMWH) and cases 3 and 5 received treatment doses of LMWH for pulmunory emboli and TTP associated stroke at presentation. Live healthy infants were delivered in all 5 cases in the third trimester (31–41 weeks). These data suggest successful pregnancy outcome is achievable in patients with a history of TTP/acute TTP with therapy based on close clinical and laboratory monitoring. Regular PEX in patients with a history of TTP and severely reduced ADAMTS 13 activity/IgG antibodies to ADAMTS 13 at the onset of pregnancy was associated with successful pregnancy outcome. Serial ADAMTS 13 activity and antibody levels during pregnancy and post-partum provided a useful adjunct to decision making on intensity of treatment.

Pregnancy-associated Thrombotic Thrombocytopenic Purpura: A Single Institution's Experience

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4675-4675
Author(s):  
Jordana Boro ◽  
Katerina Pavenski ◽  
John J. Freedman ◽  
Marie B. Garvey ◽  
Lisa K Hicks
Keyword(s):  
Plasma Exchange ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Conflicts Of Interest ◽  
Post Partum ◽  
First Trimester ◽  
Fresh Frozen Plasma ◽  
Blood Film ◽  
Intrauterine Death ◽  
Thrombocytopenic Purpura ◽  
Fresh Frozen

Abstract Abstract 4675 Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening form of microangiopathic hemolysis that can be associated with pregnancy. Although the association between TTP and pregnancy is well recognized, the presentation, natural history, and ideal treatment of TTP in this population remain poorly understood. St. Michael's Hospital is a regional referral centre for TTP in Ontario, Canada. We conducted a retrospective review of all cases of TTP referred to our pheresis unit between July 1993 and May 2010. Ninety-four cases were identified, of these, ten patients were pregnant or up to one week post-partum at the time of TTP diagnosis. Median age at presentation was 34 (29-36). Only one patient had been previously diagnosed with TTP and then developed a TTP relapse while pregnant. At presentation, all patients had thrombocytopenia (platelets: < 3 to 124 × 106/ml), anemia (hemoglobin: 60 to 99 mg/L), elevated LDH (1.3 to 12.5 × ULN), and fragmentation on blood film. ADAMTS13 was assessed in six of ten patients. It was normal in four patients, and deficient in two patients. Seven patients had neurological symptoms at presentation, one of whom suffered permanent right sided paralysis. Six patients had depressed glomerular filtration ratio (GFR) at presentation, four of these six recovered normal GFR. Hypertension was present in only one patient, and no patients had abnormal coagulation parameters at presentation. ALT was normal in six patients, mildly elevated in two patients, and unavailable in two patients. There were no patient deaths in this series. Patients were treated with a median of 17 plasma exchanges (range: 9 to 55) using either fresh frozen plasma or cryosupernatant. Eight patients achieved complete remission with plasma exchange. Two patients had refractory disease which ultimately responded to splenectomy and rituximab respectively. There were two fetal deaths, one a first trimester miscarriage, and the second an intrauterine death in the second trimester. In conclusion, in our series of pregnancy associated-TTP, there were no maternal deaths and most women responded to a short course of plasma exchange. ADAMTS13 deficiency was uncommon in our series. Nonetheless, in our series, TTP was not difficult to distinguish from preeclampsia and/or disseminated intravascular coagulation using routine laboratory parameters. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Thrombotic thrombocytopenic purpura after ChAdOx1 nCoV-19 vaccine

2021 ◽  
Vol 14 (10) ◽  
pp. e246049
Author(s):  
Hui Ping Lee ◽  
Veena Selvaratnam ◽  
Jay Suriar Rajasuriar
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Blood Film ◽  
Indian Woman ◽  
Thrombocytopenic Purpura ◽  
First Case ◽  
Immune Mediated ◽  
Peripheral Blood Film ◽  
A Disintegrin And Metalloproteinase ◽  
The Brain

A 50-year-old Indian woman presented with acute dysphasia, left upper limb numbness and thrombocytopenia 12 days after receiving the ChAdOx1 nCoV-19 vaccine (AstraZeneca/Vaxzevria). MRI of the brain was unremarkable. Microangiopathic haemolytic anaemia with thrombocytopenia was noted on her peripheral blood film. A diagnosis of thrombotic thrombocytopenic purpura (TTP) was confirmed through the findings of absent ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity and markedly raised titre of ADAMTS13 autoantibodies. Prompt treatment with plasma exchange, adjunctive steroids and rituximab was commenced. A remission of TTP was achieved and she was discharged 3 weeks after admission. While other immune-mediated conditions have been documented after receipt of the vaccine, this report highlights the first case of immune-mediated TTP diagnosed after administration of the ChAdOx1 nCoV-19 vaccine.

scholarly journals Thrombotic thrombocytopenic purpura with COVID-19 as an unforeseen complication: A case report.

2021 ◽  
Author(s):  
Muhammad Zain Mushtaq ◽  
Saad Bin Zafar Mahmood ◽  
Usman Shaikh ◽  
Syed Ahsan Ali
Keyword(s):  
Renal Function ◽  
Case Report ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Hemolytic Anemia ◽  
Peripheral Blood ◽  
Blood Film ◽  
Microangiopathic Hemolytic Anemia ◽  
Thrombocytopenic Purpura ◽  
Worsening Renal Function ◽  
Peripheral Blood Film

This article reports an association of thrombotic thrombocytopenic purpura(TTP) with COVID-19. A 49-year old male presented with fever, diarrhea and altered mentation, was found to have COVID-19. On sixth hospital day, he developed thrombocytopenia, microangiopathic hemolytic anemia with schistocytes on peripheral blood film and worsening renal function signifying TTP.

Normal Prostacyclin-Like Activity in Thrombotic Thrombocytopenic Purpura

1981 ◽  
Vol 46 (02) ◽  
pp. 571-571 ◽  
Author(s):  
M Pini ◽  
C Manotti ◽  
R Quintavalla ◽  
A G Dettori
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Thrombocytopenic Purpura
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