Splenomegaly Full Note for Internal Medicine and Pediatrics

Splenomegaly – Clinicals and Differentials

Definition

Splenomegaly is enlargement of the spleen beyond its normal size (normally not palpable below the left costal margin).

• Normal weight: ~150–200 g

• Normal length: ~11 cm

• Massive splenomegaly: Spleen palpable below the umbilicus or crossing the midline.

---

Anatomy & Physiology Summary

• Functions: Filtration of old RBCs, immune surveillance, hematopoiesis (fetal), platelet and RBC reservoir.

• Normal spleen not palpable; becomes palpable when enlarged ≥2–3×.

---

Classification of Splenomegaly

Type	Spleen size	Examples
Mild (2–3 cm)	Slight enlargement	Viral infections, hemolysis
Moderate (3–8 cm)	Reaches midway to umbilicus	Malaria, portal hypertension
Massive (>8 cm / crosses midline)	Large spleen	CML, myelofibrosis, Kala-azar

---

Pathophysiology / Mechanisms

1. Increased workload (reticuloendothelial hyperplasia)
   → Infections, hemolysis

2. Congestive (venous pooling)
   → Portal hypertension, splenic vein thrombosis

3. Infiltrative / Neoplastic
   → Leukemia, lymphoma, storage diseases

4. Immune / Inflammatory
   → SLE, rheumatoid arthritis (Felty’s syndrome)

5. Extramedullary hematopoiesis
   → Myelofibrosis, severe thalassemia

---

Causes / Differential Diagnosis of Splenomegaly

1. Infective Causes

• Acute infections:

  • Infective mononucleosis (EBV)

  • Viral hepatitis

  • Typhoid fever

  • Infective endocarditis

  • Sepsis (esp. in children)

• Chronic infections:

  • Malaria

  • Kala-azar (Visceral leishmaniasis)

  • Tuberculosis

  • Schistosomiasis

  • Brucellosis

---

2. Hematological Causes

• Hemolytic anemias

  • Thalassemia major/intermedia

  • Hereditary spherocytosis

  • Sickle cell disease (early phase)

  • Autoimmune hemolytic anemia

• Leukemias & Lymphomas

  • Chronic myeloid leukemia (CML) → massive splenomegaly

  • Chronic lymphocytic leukemia (CLL)

  • Hairy cell leukemia

  • Hodgkin / Non-Hodgkin lymphoma

• Myeloproliferative / Myelofibrotic disorders

---

3. Congestive / Portal Causes

• Portal hypertension (cirrhosis, extrahepatic portal vein obstruction)

• Splenic vein thrombosis

• Right heart failure, constrictive pericarditis

---

4. Storage / Infiltrative Disorders

• Gaucher’s disease

• Niemann–Pick disease

• Amyloidosis

• Sarcoidosis

---

5. Autoimmune / Inflammatory

• Systemic lupus erythematosus (SLE)

• Rheumatoid arthritis (Felty’s syndrome)

• Autoimmune hepatitis

---

6. Miscellaneous / Rare

• Cysts, abscess, hydatid disease

• Primary splenic tumor (hemangioma, angiosarcoma)

• Secondary metastasis (rare)

---

Massive Splenomegaly (Mnemonic: CHAMPS)

• C – Chronic myeloid leukemia

• H – Hairy cell leukemia

• A – Agnogenic myeloid metaplasia (myelofibrosis)

• M – Malaria (chronic)

• P – Portal hypertension / Kala-azar

• S – Storage diseases (Gaucher, Niemann-Pick)

---

Clinical Features

• Fullness or dragging sensation in LUQ

• Early satiety

• Pain due to infarction or capsule stretch

• Hypersplenism → Anemia, leukopenia, thrombocytopenia

• Palpable firm or hard spleen below costal margin

---

Investigations

• CBC & Peripheral smear: cytopenias, abnormal cells

• LFT, RFT

• Viral markers (EBV, hepatitis, HIV)

• Bone marrow examination

• Ultrasound / CT abdomen: spleen size, portal system, lymphadenopathy

• Serology: malaria, kala-azar (rk39), brucella

• Liver biopsy / portal venography if portal cause suspected

---

Complications

• Hypersplenism → cytopenias

• Splenic rupture (trauma or spontaneously in infections)

• Splenic infarction

• Portal hypertension

---

Management

• Treat underlying cause (infection, hematologic disorder, etc.)

• Avoid trauma / contact sports

• Splenectomy – indicated in:

  • Hypersplenism with cytopenias unresponsive to therapy

  • Hereditary spherocytosis

  • Immune thrombocytopenic purpura (refractory)

  • Splenic abscess, cyst, rupture

• Vaccinations before splenectomy: Pneumococcal, Hib, Meningococcal

---

Key Examination Tips

• Always examine in right lateral position

• Start palpation from right iliac fossa towards LUQ

• Note size, consistency, tenderness, notching, relation to costal margin

---

Summary Table

Mechanism	Common Causes
Infective	Malaria, Kala-azar, EBV
Hemolytic	Thalassemia, HS, AIHA
Neoplastic	CML, Lymphoma
Congestive	Cirrhosis, Portal HTN
Storage	Gaucher, Niemann-Pick
Autoimmune	SLE, Felty’s
Miscellaneous	Cyst, Abscess