scholarly journals Laboratory findings in hematology, clinical chemistry, and urinalysis for patients with thrombotic thrombocytopenic purpura

2020 ◽  
pp. ascls.120.002279
Author(s):  
Kathy Doig ◽  
Susan McQuiston
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Clinical Chemistry ◽  
Laboratory Findings ◽  
Thrombocytopenic Purpura

The Pathogenesis of Thrombotic Thrombocytopenic Purpura

1979 ◽  
Author(s):  
H. C. Kwaan
Keyword(s):  
Plasminogen Activator ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Degradation Products ◽  
Vascular Lesions ◽  
Local Defect ◽  
Laboratory Findings ◽  
Thrombocytopenic Purpura ◽  
Fibrin Degradation Products ◽  
Severity Of The Disease ◽  
Circulating Levels

The vascular lesions with microthrombi were studied in 12 patients with thrombotic thrombocytopenic purpura (TTP), diagnosed by the characteristic clinical and laboratory findings and confirmed histologically in each case. While defibrination was not observed, and with only minimal changes in the circulating levels of fibrinogen, fibrin degradation products and plasminogen activator, the microthrombotic lesion was invariably present. Immunofluorescent and histochemical studies indicated that both platelet and fibrin were present in the microthrombi with the platelet components dominant in many cases. Using the fibrin slide method, plasminogen activator was demonstrated in the uninvolved blood vessels but totally absent in the vessels occluded by microthrombi. in contrast, fibrinolysis is always present in the vessels afflicted with other types of thrombosis, such as the microthrombi in disseminated intravascular coagulation. Since circulating fibrinolytic activity was normal in TTP, the absence of vascular fibrinolysis is a local defect due to either inhibition by the platelet deposits or by local vascular damage. The inability of thrombolysis may explain the absence of systemic defibrination and the severity of the disease.

scholarly journals The ability of clinical and laboratory findings to predict in-hospital death in patients with thrombotic thrombocytopenic purpura in an internal and emergency medicine department

2012 ◽  
pp. 269-273
Author(s):  
Filippo Pieralli ◽  
Antonio Mancini ◽  
Alberto Camaiti ◽  
Giancarlo Berni ◽  
Carlo Nozzoli
Keyword(s):  
Emergency Medicine ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Symptom Onset ◽  
Delayed Diagnosis ◽  
Disease Process ◽  
Hemodynamic Instability ◽  
Hospital Death ◽  
Laboratory Findings ◽  
Thrombocytopenic Purpura ◽  
Lactate Levels

Introduction: Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening syndrome characterized by microangiopathic anemia, thrombocytopenia, diffuse microvascular thrombosis, and ischemia. It is associated with very low levels of ADAMTS-13. Measurement of ADAMTS-13 levels is used for diagnostic and prognostic purposes, but in every-day clinical practice, this type of analysis is not always readily available. In this retrospective study, we evaluated prognostic value of clinical and laboratory findings in patients with TTP. Materials and methods: We retrospectively investigated patients with clinically diagnosed TTP treated in a unit of Internal and Emergency Medicine (1996-2007). Clinical and laboratory findings were collected and analyzed in order to assess their ability to predict in-hospital death. Results: Twelve patients were identified (mean age 59 + 22 years; 58% were women). Five (42%) died during the hospitalization, and the variables significantly associated with this outcome were: a delay between diagnosis and symptom onset (HR 1.36; 95% CI 1.04-1.78; p < 0.05); a higher severity score (HR 1.48; 95%CI 1,23-3.86; p < 0.05); hemodynamic instability with hypotension and/or shock (HR 3.35; 95%CI 3.02-9.26; p < 0.01); a higher schistocyte count on blood smear (HR 1.84; 95%CI 1.04-3.27; p < 0.05); and higher lactate values (HR 1.85; 95%CI 1.08- 3.16; p < 0.05). Conclusions: TTP is a rare and potentially fatal disease with protean manifestations. Delayed diagnosis after symptom onset is a major determinant of poor outcome. Hypotension and shock are also prognostically unfavourable. Laboratory evidence of cardiocirculatory compromise (i.e., elevated lactate levels) and extension of the disease process (i.e., schistocyte count > 3) are predictive of in-hospital death, independently of the hemodynamic profile on admission.

scholarly journals Systemic Lupus Erythematosus Presenting as Thrombotic Thrombocytopenia Purpura: How Close Is Close Enough?

2011 ◽  
Vol 2011 ◽  
pp. 1-3 ◽  
Author(s):  
Cesar A. Perez ◽  
Nabil Abdo ◽  
Anuj Shrestha ◽  
Edgardo S. Santos
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Laboratory Findings ◽  
Thrombocytopenic Purpura ◽  
Double Stranded Dna ◽  
Immune Mediated ◽  
Healthy Female ◽  
Life Threatening

Thrombotic thrombocytopenic purpura (TTP) is an uncommon life-threatening disease characterized by microangiopathic hemolytic anemia and thrombocytopenia, commonly associated with infections, malignancy, drugs, and autoimmune diseases. We report a case of 19-year-old previously healthy female that presents with anemia and thrombocytopenia diagnosed with thrombotic thrombocytopenic purpura that was treated successfully with plasmapheresis and corticosteroids. Laboratory findings also revealed antinuclear antibodies and antibodies to double-stranded DNA. Two weeks after presentation developed inflammatory arthritis, fulfilling diagnostic criteria for systemic lupus erythematosus (SLE). Prompt diagnosis and treatment with plasma exchange and corticosteroids should be instituted as soon as the diagnosis of TTP is suspected, even if other diagnoses, including lupus, are possible. When present, the coexistence of these two etiologies can have a higher mortality than either disease alone. An underlying diagnosis of SLE should be considered in all patients presenting TTP and the study of this association may provide a better understanding of their immune-mediated pathophysiology.

Haematology

2013 ◽  
pp. 447-450
Author(s):  
Patrick Kesteven
Keyword(s):  
Chronic Disease ◽  
Iron Deficiency ◽  
Special Reference ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Lupus Anticoagulant ◽  
Laboratory Findings ◽  
Thrombocytopenic Purpura ◽  
Rheumatic Conditions ◽  
The Common

This chapter describes the common haematological abnormalities seen in rheumatic conditions with special reference to laboratory findings and diagnostic traps. The chapter is organized into sections dealing with red cell abnormalities (and the distinction between anaemia of chronic disease and iron deficiency); white cell abnormalities (neutrophilia and neutropenia); platelets (thrombocytosis, idiopathic thrombocytopenic purpura and thrombotic thrombocytopenic purpura); and finally coagulation abnormalities (lupus anticoagulant).

scholarly journals Case Report: Two Cases of Pediatric Thrombotic Thrombocytopenic Purpura Treated With Combined Therapy

2021 ◽  
Vol 9 ◽  
Author(s):  
Costanza Tripiciano ◽  
Paola Zangari ◽  
Mauro Montanari ◽  
Giovanna Leone ◽  
Laura Massella ◽  
...  
Keyword(s):  
Platelet Count ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Combined Therapy ◽  
Laboratory Data ◽  
Adamts13 Activity ◽  
Laboratory Findings ◽  
Thrombocytopenic Purpura ◽  
Life Threatening ◽  
Successful Clinical Outcome ◽  
Von Willebrand

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy caused by a severely reduced activity of the von Willebrand factor-cleaving protease ADAMTS13. Over 95% of TTPs are acquired, due to autoantibody inhibitors. In children, acquired TTP is a very rare, life-threatening disease. To date, no consensus exists on the treatment strategy of pediatric TTP. We report the cases of two pediatric patients with a diagnosis of TTP, successfully treated with a combination of various therapeutic approaches. Although the patients complained of different sets of symptoms, laboratory data showed Coombs negative hemolytic anemia, renal impairment, and low platelet count in both cases. The diagnosis of acquired TTP was supported by the PLASMIC score and confirmed by the reduction of the ADAMTS13 activity and the presence of anti-ADAMTS13 antibodies. Intravenous immunoglobulin, corticosteroids, and plasma exchange (PEX) were performed without delay. As soon as available, caplacizumab was added to the therapy, with a prompt normalization of platelet count. Nevertheless, ADAMTS13 activity was persistently low, and anti-ADAMTS13 antibodies level was high; thus, a course of rituximab was administered, with persistent normalization of laboratory findings. No adverse events were observed during the treatment. In our experience, the combined use of PEX, caplacizumab, and immunosuppressive therapy during the acute phase of the disease is safe and may have a significant impact on the prognosis with successful clinical outcome and decrease in life-threatening events.

The Pathogenesis of Thrombotic Thrombocytopenic Purpura

1979 ◽  
Author(s):  
H.C. Kwaan
Keyword(s):  
Plasminogen Activator ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Degradation Products ◽  
Vascular Lesions ◽  
Local Defect ◽  
Laboratory Findings ◽  
Thrombocytopenic Purpura ◽  
Fibrin Degradation Products ◽  
Severity Of The Disease ◽  
Circulating Levels

The vascular lesions with microthrombi were studied in 12 patients with thrombotic thrombocytopenic purpura (TTP), diagnosed by the characteristic clinical and laboratory findings and confirmed histologically in each case. While defibrination was not observed, and with only minimal changes in the circulating levels of fibrinogen, fibrin degradation products and plasminogen activator, the microthrombotic lesion was invariably present. Immunofluorescent and histochemicaL studies indicated that both platelet and fibrin were present In the mitrothrombi with the platelet components dominant In many cases. Using the fibrin slide method, plasminogen activator was demonstrated in the uninvolved blood vessels but totally absent in the vessels occluded by microthrombi. In contrast, fibrinolysis is always present in the vessels afflicted with other types of thrombosis, such as the microthrombi in disseminated intravascular coagulation. Since circulating fibrinolytic activity was normal in TTP, the absence of vascular fibrinolysis is a local defect due to either inhibition by the platelet deposits or by local vascular damage. The inability of thrombolysis may explain the absence of systemic defibrination and the severity of the disease.

scholarly journals Thrombotic Thrombocytopenic Purpura Associated with the Acquired Immune Deficiency Syndrome

1992 ◽  
Vol 3 (1) ◽  
pp. 37-41
Author(s):  
Anand Kumar ◽  
Jean Wang ◽  
David Sutton ◽  
Eric J Bow
Keyword(s):  
Immune Deficiency ◽  
Hiv Infection ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Immune Deficiency Syndrome ◽  
Acquired Immune Deficiency Syndrome ◽  
Deficiency Syndrome ◽  
Laboratory Findings ◽  
Thrombocytopenic Purpura ◽  
Immunodeficiency Virus ◽  
Acquired Immune Deficiency

A bisexual male presented with acute thrombotic thrombocytopenic purpura (TTP) in association with established acquired immune deficiency syndrome. The patient had classic clinical and laboratory findings of TTP and responded well to plasmapheresis therapy. Previously reported cases of TTP in association with human immunodeficiency virus (HIV) infection are briefly reviewed. Basic concepts in the pathogenesis of TTP are examined in reference to HIV infection.

Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome in Pregnancy: A Report of Four Case.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3970-3970
Author(s):  
Yang He ◽  
Jianmin Hu Master ◽  
Weiwen Yang ◽  
Jun Yan
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Hellp Syndrome ◽  
Good Health ◽  
Peak Serum ◽  
Laboratory Findings ◽  
Thrombocytopenic Purpura ◽  
Course Of Illness ◽  
Uremic Syndrome ◽  
The Mean

Abstract Objctive: Little information exists regarding thrombotic thrombocytopenic purpura(TTP) and hemolytic uremic syndrome(HUS) during pregnancy. We report 4 cases of TTP and HUS complicating pregnancy, with emphasis on diagnosis and management of these rare disorder. Methods: From Sep. 1988 to May. 2006, case history of four cases with either TTP(n=2) or HUS(n=2) were analyzed. Clinical and laboratory findings and maternal and fetal conditions were recorded from the medical records. Results: Of 4 cases,3 were nulliparous, one multipara. All women were in good health before pregnancy. Three in the third trimester (34w to37w), had eclampsia, 2 of whom complicated abruptio placenta. The diagnosis was made at admission as HELLP syndrome with eclampsia in 3 cases and one sespected as TTP. The laboratory analyses showed that all 4 cases were anemic(range 29.0 t0 66.0g/L) and 3 had abnormal fragmented red cells on peripheral blood smear. The platelet count ranged from 25 to 40 ×109/L The mean peak serum LDH value was 2616.75U/L(range 914 to >4000U/L),7-to 11 fold the peak serum AST and ALT values. The mental status abnormalities was in 3 cases. All cases had oliguric,2 of whom had acute onset of anuria after delivery. The mean peak serum BUN and creatinine values were 26.14±12.96mmol/L(13.1 to 37.56 mmol/L) and 647.85±347.4μmol/L(184.4 to 1027μmol/L), respectively. The renal biopsy performed on day 8 in one case with anuria, revealed findings consistent with HUS with glomerular crescents. vWF-cleaving protease was measured after plasmapheresis in case of TTP, in whom the report was normal. Treatment included fresh-frozen plasma in 3 cases except one who died before treatment. Of the 3 surviving cases, one received CRRT and plasma exchange and 2 received CRRT and hemodialysis. Of 4 cases,2 delivered vaginally, one ceasarean section, one spontaneous abortion. The course of illness was prolonged, but uneventful 24 to 25 days after treatment and discharge. All fetal death occurred before delivery. During the follow up 3 months to 16 years after discharge, one of 3 cases had another pregnancy complicating preeclamsia at 36 weeks without any other complication and the other two were all normal in renal function and BP. Conclusion: TTP and HUS complicating pregnancy is associated with high maternal mortality and morbility. The endothelial dysfunction appears to be an important factor in TTP-HUS. With the intense concern about the distinction of preeclampsia/eclampsia/HELLP syndrome from TTP/HUS, the physician must remain alert for the possibility. Improved survival after this disorder had been attributed to treatment with plasma transfusion or plasmapheresis.

Deficient ADAMTS13 Activity in Ticlopidine-Associated Thrombotic Thrombocytopenic Purpura (TTP) in Populations From Japan in 2012: Validation of Findings Initially Reported in the United States in 1998 and 2000.

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 2203-2203
Author(s):  
Charles Bennett ◽  
Masanori Matsumoto ◽  
Peter Georgantopoulos ◽  
Sony Jacob ◽  
Brianne L Dunn ◽  
...  
Keyword(s):  
United States ◽  
Board Of Directors ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Research Funding ◽  
The United States ◽  
Advisory Committees ◽  
Laboratory Findings ◽  
Thrombocytopenic Purpura ◽  
Causal Pathway ◽  
Adamts13 Deficiency

Abstract Abstract 2203 Background: Thrombotic thrombocytopenic purpura (TTP) is frequently characterized by severe ADAMTS13 deficiency, the cause of which is unknown. An important exception is ticlopidine-associated TTP (tc-TTP), the most common drug-induced TTP syndrome. In 1998, a possible association of TTP with ticlopidine was reported. In 1999, two additional series reported this association. In 2000, a fourth study reported severe A DAMTS13 deficiency among six of seven tc-TTP persons- suggesting a causal pathway. All of these reports were from the United States. Recently, we reported characteristics of 186 acquired idiopathic (ai)- TTP patients in Japan with severe ADAMTS13 deficiency, noting that clinical and laboratory findings for ai-TTP patients in Japan differed from those for cohorts of ai-TTP patients in Europe and North America- raising concern that TTP findings vary by region of the world. (PLOS One 2011) We now on report clinical and laboratory characteristics of a cohort of TTP patients from Japan with tc-TTP, and compare these findings to three cohorts of tc-TTP in the United States and one cohort of ai-TTP patients from Japan. Methods: We queried a database of thrombotic microangiopathy patients identified from a national TTP referral laboratory in Japan of cases identified between 1998 and 2008. Severe ADAMTS13 deficiency was characterized by activity levels < 5%. All tc-TTP patients and 186 of 911 ai-TTP patients in the database had severe ADAMTS13 deficiency and first onset of TTP. Comparisons were made to tc-TTP patients reported previously from the United States. Results: Characteristics of ai- TTP with severe ADAMTS13 deficiency in Japan and tc-TTP in Japan and US Conclusions: These data from Japan validate insights about tc-TTP initially proposed in 1998, 1999, and 2000 in the United States. Ticlopidine is a likely cause of TTP, the mechanism is via a cross-reactive antibody to ADAMTS13:AC resulting in formation of an ADAMTS13:INH, and therapeutic plasma exchange is necessary for treatment. Disclosures: Ortel: Eisai: Research Funding; Glaxo SmithKline: Research Funding; Pfizer: Research Funding; Instrumentation Laboratory, Inc: Consultancy, Research Funding; Boehringer Ingelheim: Consultancy. Fujimura:Baxter BioScience: Membership on an entity's Board of Directors or advisory committees; Alexion Pharma: Membership on an entity's Board of Directors or advisory committees.

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