SAT-418 RENAL OUTCOMES IN PATIENTS WHO HAD PLASMA EXCHANGE FOR MICROANGIOPATHIC HAEMOLYTIC ANAEMIA FOLLOWING VIPER BITES - A STUDY IN SRI LANKA

BACKGROUND AND AIM: Adult, non-infective, haemolytic-uremic syndrome (HUS) although a rare disease in itself, has a high likelihood of occurrence in pregnancy and immediate post partum period. It is an important differential diagnosis in the evaluation of thrombotic microangiopathies. Patients with post-partum HUS display a classical triad of microangiopathic haemolytic anaemia, acute nephropathy and thrombocytopenia. I hereby present a case of post partum HUS treated with therapeutic plasma exchange (TPE) MATERIAL AND METHODS: A total of six sessions of TPE were performed daily, three sessions for consecutive days and remaining three sessions were performed on alternate days. All the procedures were carried out with Haemonetics MCS+ exchanging one plasma volume using fresh frozen plasma and saline as replacement fluid. Haemodialysis was started and four sessions were carried out on alternate days. RESULT: A 37 year old, 85 kg female, G2 P1, underwent emergency LSCS because of foetal distress at 38 weeks of pregnancy. Post surgery she developed decreasing urine output, anuria ensued. Emergency therapeutic plasma exchange was carried out within 24 hours of diagnosis. It could be found that with TPE, patient had improvement in renal function, decrease in LDH levels and increase in platelet count. Patient had sustained remission and discontinuation of haemodialysis. CONCLUSION: HUS is a disorder with high mortality and long term morbidity, if prompt treatment is not instituted. The decision to intervene with plasma exchange should be based upon the severity of thrombocytopenia, microangiopathic haemolytic anaemia and neurological abnormalities, even if the diagnosis and nomenclature is uncertain. Improved survival after this disorder has been attributed to aggressive treatment with plasma exchange therapy.

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Thrombotic Thrombocytopenic Purpura: Etiopathogenesis, Diagnostics and Basic Principles of Treatment

Serbian Journal of Experimental and Clinical Research ◽

10.1515/sjecr-2016-0026 ◽

2017 ◽

Vol 18 (1) ◽

pp. 61-68

Author(s):

Željko Todorović ◽

Milena Jovanovic ◽

Dusan Todorovic ◽

Dejan Petrovic ◽

Predrag Djurdjevic

Keyword(s):

Plasma Exchange ◽

Thrombotic Thrombocytopenic Purpura ◽

Haemolytic Anaemia ◽

Signs And Symptoms ◽

Genetic Mutation ◽

Clinical Syndrome ◽

Main Function ◽

Adamts13 Activity ◽

Thrombocytopenic Purpura ◽

Microangiopathic Haemolytic Anaemia

Abstract Thrombotic thrombocytopenic purpura (TTP) is a clinical syndrome that manifests with thrombocytopenia, microangiopathic haemolytic anaemia and symptoms and signs of kidney and brain damage, but it rarely involves other organs. The main pathophysiological cause of TTP is diminished metalloproteinase ADAMTS13 activity; the main function of ADAMTS13 is to degrade large multimers of the von Willebrand factor. Diminished activity of ADAMTS13 is caused either by a genetic mutation in the gene that codes ADAMTS13 (congenital TTP) or by antibodies that block ADAMTS13 enzyme activity or accelerate the degradation of ADAMTS13 (acquired TTP). Clinically, TTP presents most frequently with signs and symptoms of brain and kidney damage with concomitant haemorrhagic syndrome. TTP is suspected when a patient presents with a low platelet count, microangiopathic haemolytic anaemia (negative Coombs tests, low haptoglobine concentration, increased serum concentration of indirect bilirubin and lactate dehydrogenase, increased number of schysocytes in peripheral blood) and the typical clinical presentation. A definitive diagnose can be made only by measuring the ADAMTS13 activity. The differential diagnosis in such cases includes both typical and atypical haemolytic uremic syndrome, disseminated intravascular coagulation, HELLP syndrome in pregnant women and other thrombotic microangiopathies. The first line therapy for TTP is plasma exchange. In patients with acquired TTP, in addition to plasma exchange, immunosuppressive medications are used (corticosteroids and rituximab). In patients with hereditary TTP, the administration of fresh frozen plasma is sometimes required.

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Thrombotic Thrombocytopenic Purpura: Is Plasma Exchange Enough? A Fifteen Year Australian Experience at the Royal Melbourne Hospital.

Blood ◽

10.1182/blood.v108.11.3991.3991 ◽

2006 ◽

Vol 108 (11) ◽

pp. 3991-3991

Author(s):

Ashley P. Ng ◽

Natasha Frawley ◽

Chris Hogan ◽

Andrew Grigg ◽

Solomon Cohney ◽

...

Keyword(s):

Platelet Count ◽

Plasma Exchange ◽

Thrombotic Thrombocytopenic Purpura ◽

Haemolytic Anaemia ◽

Immunosuppressive Agents ◽

Neurological Dysfunction ◽

Treatment Intensification ◽

Royal Melbourne Hospital ◽

Thrombocytopenic Purpura ◽

Microangiopathic Haemolytic Anaemia

Abstract Introduction Thrombotic Thrombocytopenic Purpura (TTP) is a cause of microangiopathic-haemolytic anaemia and thrombocytopenia, associated with renal and neurological dysfunction with thrombotic complications causing significant morbidity and mortality. Methods A restrospective single-institution analysis of patients with TTP treated between 1990–2005. Renal or bone marrow transplantation patients were excluded. Results Forty patients were identified. Aetiology was idiopathic 75% (n=30), connective tissue disease-related 12.5% (n=5), malignancy-related 5% (n=2) and pregnancy-related 7.5% (n=3). Presenting features: neurological 62.5% (n=25), renal impairment (creatinine>0.11 mmol/L) 76% (n=28), microangiopathic-haemolytic anaemia 97.5% (n=39) and thrombocytopenia 100% (mean platelet-count 42x10^9/L). Mean Hb 93 g/L and mean Lactose dehydrogenase (LD) 2517 U/L (<420). 38 patients received up-front single plasma-volume plasma-exchange using fresh-frozen plasma (median 11 exchanges). All received steroids. Complete-response (CR) (normalisation of platelet count, LD, neurological examination and rising Hb), was achieved in 79% (n=30) following plasma-exchange of whom 43% (n=13) relapsed (median 14.5 days from therapy-cessation): eleven achieved CR2 with further therapy and two died from TTP-related complications. Partial-response (PR) was obtained in 7.9% (n=3) with up-front plasma-exchange: one remained in PR with prostate-cancer treatment and two relapsed: one achieved CR and one sustained-PR with further therapy. 13% (n=5) were refractory to plasma-exchange: four died from TTP-related complications and one achieved CR with treatment intensification. Therapeutic intensification included steroids (n=34), vincristine (n=7), intravenous immunoglobulin (n=6), Rituximab (n=3) and splenectomy (n=6). Conclusion TTP is associated with a significant relapse-rate (43%) and TTP-related mortality (16%) despite plasma-exchange. Delineation of patients at high risk of relapse following CR will potentially allow targetted treatment intensification. Treatment intensification is also clearly required for patients with refractory TTP and consideration may be given to other immunosuppressive agents, such the chimeric monoclonal anti-CD20 antibody, Rituximab, as a steroid-sparing agent and to potentially avoid splenectomy. We propose a central TTP registry is developed within Australia such that therapeutic strategies can be systematically evaluated in a multicentre setting.

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