What is the management for a postpartum patient with refractory anemia, elevated Lactate Dehydrogenase (LDH), and thrombocytopenia after an emergency Lower Segment Caesarean Section (LSCS)?

Management of Postpartum Refractory Anemia with Elevated LDH and Thrombocytopenia

This patient requires immediate evaluation for thrombotic microangiopathy (TMA), specifically atypical hemolytic uremic syndrome (aHUS) or HELLP syndrome, with urgent ADAMTS13 testing and consideration for complement blockade therapy if aHUS is confirmed. 1

Immediate Diagnostic Workup

The triad of non-immune hemolytic anemia, thrombocytopenia, and elevated LDH in the postpartum period mandates urgent investigation for TMA. 1

Essential Laboratory Tests (Stat)

• Direct Coombs test to confirm non-immune hemolysis 1
• Haptoglobin levels (expect low/undetectable) and indirect bilirubin (expect elevated) 1
• Peripheral blood smear for schistocytes (>1% supports TMA, but absence does not exclude it) 1
• ADAMTS13 activity level to distinguish thrombotic thrombocytopenic purpura (TTP) from aHUS—severely deficient (<10 IU/dL) indicates TTP 1
• Renal function (creatinine, urinalysis for hematuria/proteinuria) as renal involvement is common in aHUS 1
• Coagulation studies (PT, APTT, fibrinogen) to assess for DIC or HELLP-related coagulopathy 1

Critical Distinction: HELLP vs. aHUS vs. TTP

HELLP syndrome typically presents with hemolysis, elevated liver enzymes (AST >200 U/L, ALT >159 U/L), and low platelets (<100,000/mm³), usually resolving within days postpartum. 1 However, if thrombocytopenia is worsening 2 days postpartum despite delivery, consider complement-mediated TMA (aHUS). 1

Atypical HUS should be suspected when:

• Thrombocytopenia and hemolysis persist or worsen beyond 72 hours postpartum 1
• Renal involvement is present (hematuria, proteinuria, rising creatinine) 1
• ADAMTS13 activity is >10% 1

TTP is characterized by ADAMTS13 activity <10% and may present with neurological symptoms (seizures, altered mental status). 1, 2

Immediate Management Algorithm

If HELLP Syndrome is Suspected (Most Likely Given Timing)

1. Continue supportive transfusion therapy as already initiated 1

   • Maintain hemoglobin >7-8 g/dL with PRBCs 1
   • Transfuse platelets if count <50,000/mm³ and ongoing bleeding or <20,000/mm³ prophylactically 1
   • Administer FFP if PT/APTT prolonged or fibrinogen <2 g/L 1

2. Monitor for progression with serial labs every 6-12 hours (CBC, LDH, liver enzymes, creatinine) 1

3. Blood pressure control if hypertensive (>160/90 mmHg) with IV hydralazine or labetalol 1

4. Expect resolution within 48-96 hours postpartum; platelet count typically normalizes by day 6-11 1

If aHUS is Suspected (Worsening After 48-72 Hours)

1. Do NOT delay treatment waiting for genetic testing—aHUS is a clinical diagnosis 1

2. Initiate complement blockade with eculizumab (anti-C5 monoclonal antibody) immediately if:

   • Thrombocytopenia worsening beyond 72 hours postpartum 1
   • Rising creatinine or oliguria 1
   • Persistent hemolysis (elevated LDH, low haptoglobin) 1

3. Meningococcal vaccination is required before eculizumab, but do NOT delay treatment—give antibiotic prophylaxis (penicillin or macrolide) concurrently 1

4. Plasma exchange may be considered as bridge therapy while awaiting eculizumab, though less effective than in TTP 1

If TTP is Confirmed (ADAMTS13 <10%)

1. Urgent plasma exchange (TPE) is life-saving and should begin within hours of diagnosis 2
2. Avoid platelet transfusions unless life-threatening bleeding, as they may worsen thrombosis 2

Critical Pitfalls to Avoid

• Do not assume HELLP will resolve spontaneously if thrombocytopenia continues falling after 48-72 hours—this suggests aHUS 1
• Do not wait for ADAMTS13 results to initiate plasma exchange if TTP is strongly suspected clinically 1
• Do not transfuse platelets prophylactically in TTP without active bleeding 2
• Do not overlook vitamin B12 deficiency as a rare mimic of TMA—check B12, methylmalonic acid if macrocytosis or neurological symptoms present 3, 4
• Erythropoietin is NOT indicated in acute hemolytic anemia and will not address the underlying TMA 1

Monitoring and Escalation

• ICU-level monitoring for first 24-48 hours with continuous vital signs, hourly urine output, and serial labs 1
• Central venous access for volume assessment if oliguria develops 1
• Neurological examination every 4-6 hours; obtain brain MRI if seizures, altered mental status, or focal deficits occur (look for basal ganglia hyperintensities on FLAIR/T2) 1
• Hematology and nephrology consultation immediately if aHUS or TTP suspected 1

Prognosis and Follow-Up

HELLP syndrome typically resolves completely within 1 week postpartum with supportive care alone. 1

Atypical HUS has high mortality (up to 25%) without complement blockade therapy, but excellent outcomes with eculizumab. 1 Long-term complement blockade may be required depending on genetic mutations. 1

TTP has >90% mortality untreated but >80% survival with prompt plasma exchange. 2