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Thrombotic Microangiopathy (TMA)

A syndrome characterized by microvascular thrombosis, hemolytic anemia, and thrombocytopenia.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dahiliye (İç Hastalıkları) department. Book Appointment →

What is Thrombotic Microangiopathy (TMA)?

Thrombotic microangiopathy (TMA) is a clinical syndrome group characterized by platelet thrombi formation in microcirculation (arterioles and capillaries) leading to microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and end-organ ischemia. The classic triad is hemolytic anemia + thrombocytopenia + end-organ damage.

Main types: TTP (thrombotic thrombocytopenic purpura, ADAMTS13 deficiency, autoimmune in adults), STEC-HUS (Shiga toxin E. coli hemolytic uremic syndrome, dominant in children, kidney damage), aHUS (atypical HUS, complement system activation), secondary TMA (drugs, pregnancy, malignancy, transplantation, autoimmune diseases).

Diagnosis: hemoglobin <10 g/dL, platelet <150,000, schistocytes on blood smear (>1%), elevated LDH, undetectable haptoglobin, indirect bilirubin elevation, negative Coombs. ADAMTS13 activity <10% is diagnostic for TTP. Plasma exchange should be started emergently in TTP and aHUS suspicion (mortality 90% without treatment, 10-20% with treatment).

Symptoms

Severe weakness, fatigue, pallor (anemia)
Jaundice (hemolysis)
Petechiae, purpura, mucosal bleeding (thrombocytopenia)
Acute renal failure (hematuria, proteinuria)
Neurologic findings: confusion, headache, seizures, coma (TTP)
Fever
Abdominal pain, diarrhea (STEC-HUS, often bloody)
Cardiac findings: chest pain, arrhythmia, troponin elevation
Pulmonary involvement: dyspnea, hypoxia

Risk Factors

TTP: female sex, African ethnicity, autoimmune diseases (lupus)
STEC-HUS: undercooked meat, raw milk, contact with pediatric patients
aHUS: genetic complement mutations (CFH, CFI, MCP)
Pregnancy and postpartum
Drugs: quinine, ticlopidine, clopidogrel, calcineurin inhibitors
Cancer and chemotherapy
Bone marrow and solid organ transplantation
HIV, malignant hypertension
Family history (genetic forms)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Sudden severe weakness, jaundice
  • Widespread bruising, petechiae, mucosal bleeding
  • Sudden confusion, focal neurologic findings, seizure
  • Sudden onset oliguria, hematuria
  • Bloody diarrhea (especially in children)
  • Postpartum unexplained bleeding and weakness
  • Unexplained fever and acute renal failure

Treatment Methods

01
EMERGENT plasma exchange (TPE) - within 4-8 hours of suspicion
02
FFP infusion (until plasma exchange is started)
03
ADAMTS13 activity and inhibitor measurement
04
Caplacizumab (TTP - rapid platelet response)
05
Rituximab (TTP - immunosuppression)
06
Eculizumab (aHUS - C5 inhibitor)
07
Steroid (immune-mediated TMA)
08
Supportive care: dialysis, mechanical ventilation, transfusion
09
Folic acid supplementation
10
Long-term follow-up: ADAMTS13, complement panel, genetic testing

Which Department to Visit?

You can visit our Dahiliye (İç Hastalıkları) department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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