Editing Leukaemia

Neoplasm of blood-forming tissues

== '''Chronic lymphocytic leukaemia (CLL)''' ==

* 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
**

{| class="wikitable"
|'''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)''' ==

* 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''' ==

* 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.

[[Category:Haematology]]