Diagnosis: Hepatosplenic Schistosomiasis with Hypersplenism
This 36-year-old farmer presents with classic hepatosplenic schistosomiasis mansoni, confirmed by ultrasound findings of diffuse liver parenchymal disease with features of schistosomiasis and marked splenomegaly (23 cm), along with pancytopenia (anemia, leukopenia, thrombocytopenia) consistent with hypersplenism. 1, 2
Clinical Presentation Analysis
The constellation of findings strongly supports schistosomiasis:
- Right upper quadrant pain with fever in a farmer (occupational freshwater exposure risk) presenting with hepatosplenomegaly is characteristic of hepatosplenic schistosomiasis 1
- Marked splenomegaly (23 cm) with palpable liver edge and spleen on examination, confirmed by ultrasound 1, 3
- Pancytopenia pattern: Hemoglobin 8.1-8.5 g/dL, WBC 1.64-1.94 ×10³/µL, platelets 40-70 ×10³/µL—this triad is pathognomonic for hypersplenism secondary to portal hypertension from schistosomal periportal fibrosis 3, 4
- Gallbladder wall thickening without cholecystitis suggests portal hypertension-related changes 1
Hematologic Abnormalities Explained
The severe cytopenias result from hypersplenism, not bone marrow failure:
- Thrombocytopenia (40-70 ×10³/µL) correlates directly with spleen size in hepatosplenic schistosomiasis, with statistically significant association (p=0.004) 3, 4
- Leukopenia (1.64-1.94 ×10³/µL) also correlates with splenomegaly (p=0.046) 3, 4
- Anemia (Hgb 8.1-8.5 g/dL) occurs in 36.3% of hepatosplenic schistosomiasis patients, related to hypersplenism and possible gastrointestinal blood loss 3, 4
- Important caveat: EDTA-dependent pseudothrombocytopenia occurs in 8.92% of hepatosplenic schistosomiasis patients versus 0% in healthy controls (p<0.0285), so platelet counts should be verified by manual blood smear examination 5
Diagnostic Workup Required
Immediate testing needed:
- Stool microscopy (three samples on different days) using Kato-Katz thick smear technique to identify Schistosoma mansoni eggs 1
- Schistosoma serology (IgG antibodies), though may be negative in chronic established disease 1, 2
- Liver function tests to assess hepatic synthetic function—expect elevated transaminases and alkaline phosphatase with preserved albumin initially 3, 4
- Coagulation studies (PT/INR, aPTT, fibrinogen, D-dimer) as INR averages 1.42 and aPTT 37.9 seconds in hepatosplenic schistosomiasis versus 1.04 and 30.5 seconds in controls 4
- Upper endoscopy to evaluate for esophageal varices given portal hypertension, though only 4.5% present with hemorrhage initially 3
Management Protocol
Antiparasitic treatment:
- Praziquantel 40 mg/kg as a single oral dose, repeated at 6-8 weeks is the treatment of choice for schistosomiasis 1, 2
- Alternative: Oxamniquine (used in 35.4% of patients in Brazilian cohorts) 3
- Do not delay treatment waiting for confirmatory serology if clinical suspicion is high, as serology may take up to 6 months to become positive 2
Supportive management for portal hypertension:
- Propranolol (non-selective beta-blocker) for primary prophylaxis of variceal bleeding—used in 65% of hepatosplenic schistosomiasis patients 3
- Omeprazole (proton pump inhibitor) for gastroprotection—used in 90% of patients 3
- Endoscopic surveillance with variceal band ligation or sclerotherapy as needed—92.9% of patients require endoscopic treatment, with 85% receiving sclerotherapy and 62.5% elastic band ligation 3
Alcohol-Related Liver Disease Consideration
Given heavy alcohol consumption history, concurrent alcohol-related liver disease must be addressed:
- AST/ALT ratio should be calculated—ratio >1.5-2.0 suggests alcoholic hepatitis, though this patient's presentation is more consistent with schistosomiasis 1
- Alcohol abstinence monitoring using urinary ethyl glucuronide (uETG) or hair ethyl glucuronide (hEtG) with cut-offs: abstinence <7 pg/mg, chronic excessive consumption >30 pg/mg 1
- Alcohol cessation is mandatory as continued drinking will accelerate liver fibrosis progression 1
Critical Pitfalls to Avoid
- Do not attribute pancytopenia to bone marrow pathology without first recognizing hypersplenism from portal hypertension 3, 4
- Verify platelet counts manually on blood smear, as EDTA-dependent pseudothrombocytopenia occurs in 8.92% of hepatosplenic schistosomiasis cases and automated counts may be falsely low 5
- Do not perform sodium citrate tube collection as an alternative, as platelet clumping still occurs in citrate tubes in schistosomiasis patients 5
- Perform automated platelet counts immediately (within 20 minutes) after blood collection in EDTA tubes to minimize artifact 5
- Do not assume absence of esophageal hemorrhage means no varices—92.9% require endoscopic intervention despite only 4.5% presenting with bleeding 3
Prognosis and Follow-up
Multidisciplinary outpatient follow-up with combined endoscopic and drug treatments is effective at preventing bleeding complications in 95.5% of patients when implemented systematically 3