Massive Splenomegaly & Haematemesis: Diagnosis

(i) Five Differential Diagnoses of Massive Splenomegaly

• Chronic Liver Disease with Portal Hypertension: This is a common cause, particularly cirrhosis (alcoholic, viral hepatitis B/C, non-alcoholic steatohepatitis). Portal hypertension leads to congestive splenomegaly.
• Haematological Malignancies: Leukaemias (Chronic Myeloid Leukaemia - CML, Acute Myeloid Leukaemia - AML), Lymphomas (Hodgkin’s and Non-Hodgkin’s), and Myeloproliferative Neoplasms (MPNs) like Primary Myelofibrosis can cause significant splenic enlargement.
• Infections: Chronic infections like Malaria (especially *Plasmodium falciparum*), Tuberculosis, Visceral Leishmaniasis (Kala-azar), and Brucellosis can lead to massive splenomegaly.
• Storage Diseases: Gaucher’s disease, Niemann-Pick disease, and other lysosomal storage disorders can cause splenic infiltration and enlargement.
• Splenic Cysts: Although less common, large splenic cysts (epidermoid, pseudocysts) can result in massive splenomegaly.

(ii) Most Likely Diagnosis in this Patient

Given the presentation of haematemesis alongside massive splenomegaly, the most likely diagnosis is Cirrhosis with Portal Hypertension. The haematemesis suggests bleeding from esophageal varices, a common complication of portal hypertension secondary to cirrhosis. While haematological malignancies and infections are possibilities, the acute presentation with bleeding makes cirrhosis the most probable cause.

(iii) Investigations Warranted

• Complete Blood Count (CBC) with Peripheral Smear: To assess for anaemia, thrombocytopenia, and abnormal cells suggestive of haematological malignancy.
• Liver Function Tests (LFTs): To evaluate liver damage and assess the degree of hepatic dysfunction (bilirubin, ALT, AST, albumin, prothrombin time).
• Coagulation Profile: PT/INR, aPTT to assess clotting factors and bleeding risk.
• Viral Hepatitis Serology: HBsAg, anti-HCV, anti-HAV IgM to identify viral hepatitis as the cause of cirrhosis.
• Ultrasound Abdomen with Doppler: To confirm splenomegaly, assess liver morphology (cirrhosis), and evaluate portal vein and splenic vein for patency and portal hypertension.
• Upper Gastrointestinal Endoscopy (UGIE): To identify the source of haematemesis (esophageal varices, gastritis, ulcers) and assess the severity of varices.
• CT Scan Abdomen: If ultrasound is inconclusive or to further evaluate the liver and spleen, and rule out other causes like splenic masses.
• Bone Marrow Aspiration and Biopsy: If haematological malignancy is suspected based on CBC and peripheral smear.
• Leishmaniasis testing: rK39 dipstick test and ELISA for visceral leishmaniasis, especially if the patient is from an endemic area.

(iv) Initial Management in Emergency

1. Resuscitation: Assess airway, breathing, and circulation (ABCs). Administer oxygen if needed.
2. Intravenous Access: Establish two large-bore IV lines for fluid resuscitation and blood transfusion.
3. Fluid Resuscitation: Initiate crystalloid infusion (Normal Saline or Ringer’s Lactate) to restore intravascular volume.
4. Blood Transfusion: Transfuse packed red blood cells if haemoglobin is significantly low (e.g., <7 g/dL) or if the patient is hemodynamically unstable.
5. Pharmacological Management:
   • Octreotide: Administer octreotide (a somatostatin analogue) to reduce portal pressure and decrease variceal bleeding.
   • Terlipressin: Can be used as an alternative to octreotide.
   • Proton Pump Inhibitors (PPIs): Administer IV PPIs (e.g., pantoprazole) to reduce gastric acidity and promote clot formation.
6. Endoscopic Intervention: Urgent UGIE should be performed to confirm the source of bleeding and initiate endoscopic therapy (banding or sclerotherapy) for esophageal varices.
7. Monitoring: Continuous monitoring of vital signs, oxygen saturation, and urine output.