Makindo Medical Notes"One small step for man, one large step for Makindo" |
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Haematological malignancies are best understood by lineage (myeloid vs lymphoid) and tempo (acute vs chronic). This dual framework is essential for exams and clinical reasoning. Both groups represent uncontrolled clonal proliferation, but differ in cell lineage, pathophysiology, clinical presentation, and prognosis.
| Type | Acute 🔥 (rapid onset, aggressive) | Chronic 🕐 (slower course, indolent or progressive) |
|---|---|---|
| Myeloid Lineage 🧬 |
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| Lymphoid Lineage 🧪 |
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| Feature | Myeloproliferative Disorders (MPDs) 🧬 | Lymphoproliferative Disorders (LPDs) 🧪 |
|---|---|---|
| Cell line affected | Myeloid stem cells → ↑ granulocytes, platelets, RBCs | Lymphoid lineage → ↑ B-cells, T-cells, NK cells |
| Driver mutations | BCR-ABL (CML), JAK2 (PV, ET, MF), CALR, MPL | Genetic lesions affecting apoptosis (e.g., TP53, BCL2); viral triggers (EBV, HTLV-1, HIV) |
| Clinical features | Splenomegaly, thrombosis, bleeding, constitutional symptoms, risk of AML transformation | Painless lymphadenopathy, B symptoms (fever, night sweats, weight loss), cytopenias from marrow infiltration |
| Investigations | FBC: raised counts, bone marrow biopsy (hypercellular), mutation testing | FBC: lymphocytosis or cytopenias, excisional node biopsy, flow cytometry, staging CT/PET |
| Management | Imatinib (CML), JAK inhibitors, hydroxycarbamide, phlebotomy (PV), stem cell transplant in selected | Watch-and-wait (indolent), chemo-immunotherapy (R-CHOP, ABVD), rituximab, stem cell transplant if refractory |
| Prognosis | Chronic course with risk of AML transformation; survival improved with TKIs | Variable: Hodgkin’s lymphoma curable in >80%; CLL/NHL often indolent but incurable |