Related Subjects:
|Leukaemias in General
|Acute Promyelocytic Leukaemia
|Acute Myeloblastic Leukaemia (AML)
|Acute Lymphoblastic Leukaemia (ALL)
|Chronic Lymphocytic leukaemia (CLL)
|Chronic Myeloid Leukaemia (CML)
|Hairy Cell Leukaemia
|Differentiation syndrome
|Tretinoin (All-trans-retinoic acid (ATRA) )
|Haemolytic anaemia
|Immune(Idiopathic) Thrombocytopenic Purpura (ITP)
🧬 Unlike most other leukaemias, Hairy Cell Leukaemia (HCL) shows minimal lymph node involvement.
Instead, malignant cells preferentially infiltrate the bone marrow, spleen, and liver.
ℹ️ About
- 🎗️ Rare, chronic B-cell lymphoproliferative disorder (~2% of all leukaemias)
- 👨 Male predominance (~4:1); median age at diagnosis ≈ 50 years
- 🌍 More common in European and Mediterranean ancestry
🧬 Aetiology
- ☢️ Environmental exposures: radiation, pesticides, and organic solvents
- 🧪 Strong association with BRAF V600E mutation (diagnostic hallmark, targetable)
🩺 Clinical Features
- 📉 Cytopenias: anaemia, neutropenia, thrombocytopenia → fatigue, infections, bruising
- 🫀 Splenomegaly in ~90% → abdominal fullness, early satiety, left upper quadrant pain
- ⚖️ Weight loss, malaise, recurrent infections
- 🌿 Lymphadenopathy is rare (helps distinguish from CLL/NHL)
🔎 Investigations
- 🧪 FBC: pancytopenia; classically severe monocytopenia
- 🔬 Blood film: “hairy cells” (B-lymphocytes with fine cytoplasmic projections)
- 🦴 Bone marrow: often fibrotic (“dry tap”), biopsy shows hairy cells
- 📌 Immunophenotype: CD19+, CD20+, CD25+, CD103+, Annexin A1+ (characteristic)
- 🧬 BRAF V600E mutation testing confirms diagnosis and guides targeted therapy
💊 Management
- 🌟 First-line chemotherapy:
– Cladribine (2-CdA) → high complete remission rates
– Pentostatin (deoxycoformycin) as alternative
- ♻️ Relapse common → repeat purine analogue therapy often effective
- 💉 Targeted therapy: BRAF inhibitors (e.g. vemurafenib) for refractory cases
- 🛡️ Supportive care: infection prophylaxis, G-CSF if neutropenic, transfusion support if required
📚 References
