Leukaemia
Neoplasm of blood-forming tissues
Chronic lymphocytic leukaemia (CLL)
[edit | edit source]- Epidemiology
- Most common leukaemia in the western world
- Slight male predominance
- Mainly after the age of 50 years
- Pathophysiology
- Disease of B lymphocytes
- Progressive accumulation of relatively morphologically normal, mature but functionally incompetent lymphocytes
- Staging
- Use Rai system with Binet modification - correlates with survival
| Risk (Binet staging) | Stage | Description |
| Low | 0 | Lymphocytosis in blood or bone marrow |
| Intermediate | I | Lymphocytosis + enlarged lymph nodes |
| II | Lymphocytosis + enlarged liver or spleen with or without lymphadenopathy | |
| High | III | Lymphocytosis + anemia (Hgb <11 g/dL) with or without enlarged liver, spleen, or lymph nodes |
| IV | Lymphocytosis + thrombocytopenia (platelet count <100,000/microL) with or without anemia or enlarged liver, spleen, or lymph nodes |
- Treatment
- Reserved for fit patients with advanced-stage disease
Chronic myelogenous leukaemia (CML)
[edit | edit source]- Epidemiology
- Can occur from childhood to old age
- Pathophysiology
- Neoplastic transformation of myeloid elements
- Progressive replacement of the bone marrow with mature-appearing neoplastic myeloid cells
- Gold standard diagnosis with chromosomal marker for Philadelphia chromosome (22q11.2) - BCR-ABL1 oncoprotein expression, which accelerates cell division and inhibits DNA repair
- Presentation
- Can be asymptomatic
- Commonly fever, fatigue, malaise, pancytopaenia, and occasionally splenomegaly
- Leukocytosis up to 100
- Treatment
- Target the oncoprotein with tyrosine kinase inhibitors and chemotherapy
- Bone marrow transplant is an option
- No survival benefit to splenomegaly, but can be done for symptoms
Hairy cell leukaemia
[edit | edit source]- Epidemiology
- Rare disease, about 2% of adult leukaemias
- Pathophysiology
- Originates with B lymphocytes
- Ruffling of cell membrane - this ruffling gives the name due to appearance under microscope
- Presentation
- Splenomegaly, pancytopaenia, and neoplastic mononuclear cells in the peripheral blood and bone marrow
- Behaves like a chronic leukaemia - many patients can achieve clinical remission and near-normal lifespan
- Treatment
- Splenectomy indicated with symptomatic splenomegaly - most show improvement after splenectomy. Diffusely involved bone marrow means less likely to respond to splenectomy.