Bone marrow failure

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  • Created by: MazzaW
  • Created on: 06-12-19 13:59

Pancytopenia

Anaemia, neutropenia and thrombocytopenia

Neutropenia: very dangerous, especially if febrile. Classified according to neutrophil count (mild = 1.0-1.5, moderate = 0.5-1.0, severe = 0.1-0.5). Major risk if febrile neutropenia/neutropenic sepsis.

Thrombocytopenia: classified according to platelet count (mild = 50-100, moderate = 20-50, severe = <20). Causes predisposition to bleeding (bruising, petechiae, risk of intracranial/GI haemorrhage)

Ix: Hx, Ex, drug Hx, hepato/splenomegaly, blood film, reticulocytes count, B12/folate (megaloblastic anaemia- should always check this first as it is easily correctable), abdo USS/CT (organomegaly, occult bleeds), BM biopsy (hypocellular = aplastic anaemia, hypercellular = marrow infiltration- BM completely replaced by cancer)

Infiltration with cancer may be from a haematological cancer or it may be metastatic from breast/prostate/lung/renal/thyroid/melanoma

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Aplastic anaemia

Rare, occasionally congenital. 

60% cases are idiopathic (possibly autoimmune?)

Other 40% due to drugs: benzene, gold/penicillamine, chemo (usually reversible), sulphonamides

Rx: withdraw cause, supportive care (blood/platelet transfusions, Abx), immunosuppression (if autoimmune), stem cell transplantation if particularly severe

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Myelofibrosis

Fibrotic condition where the BM is scarred over by collagen (replaced by scar tissue)

Body attempts to compensate by producing cells in liver and spleen -> hepatosplenomegaly

Only treatment is BM transplant

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Acute leukaemia

Commonest reason for BM failure. Cancer cells are undifferentiated blasts.

Presentation: BM failure, often systemically unwell, rapid onset and progression, often flu-like illness, sweats, wt loss, anorexia. May have infiltration of other sites (CNS- leukaemic meningitis, testes- painless swelling, gums, skin, thymus- mediastinal mass on CXR, nodes)

Prognostic factors: age (younger=better), sex (females do better), cytogenetics, remission/speed of remission, relapse, blast count in blood, extramedullary disease

Causes: genetic predisposition, haematological disease, irradiation, chemicals (including chemo)

Dx: FBC (pancytopenia), deranged clotting, BM biopsy (diagnostic), tissue biopsy (if infiltration), chromosomal analysis

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Acute leukaemia treatment

Rapidly fatal if untreated- need immediate intensive chemo (sperm banking 1st), treat infection, hydration, transfusion, allopurinol (high risk of tumour lysis syndrome), may need intrathecal chemo/transplant

Common S/E: N+V, BM failure (gets worse before it gets better), hair loss, tiredness.

Long-term: infertility, short stature, loss of intellect, lifelong increased Ca risk, psychological, cardiac risk

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Chronic leukaemia

Cancer cells look like normal counterparts (differentiated)

BM function fairly normal at presentation. Indolent presentation.

Patients rarely unwell- may not need treatment.

Chronic illness: patients can live with the disease for many years

Myeloid leukaemias: cancer cells derived from neutrophils/RBCs/platelet precursors. Prevalence increases with age.

Lymphoid leukaemias: cancer cells derived from immune cells/lymphocytes. Most common cancer of childhood.

CML: due to Philadelphia chromosome (9:22 BCR:ABL translocation), treat with tyrosine kinase inhibitors

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Myelodysplastic syndrome

Progressive BM failure- disease of the elderly.

Difficult to treat. 

Pre-leukaemic. Can be terminal either from BM failure or from transformation to acute leukaemia.

Can be induced by chemo/radiotherapy.

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