Acute Myeloid Leukemia (AML)

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Definition

neoplastic proliferation of myeloid blast cells

Epidemiology

rapid progression

- death ~2/12 if untreated

- ~20% 3-yr survival after Rx

commonest acute leukemia of adults

- incidence increases w age

Risk Factors

can be LT complication of chemotherapy

also associated w myelodysplastic states, radiation, syndromes (eg. Down's)

Morphological classification

AML w recurrent genetic abnormalities

AML multi-lineage dysplasia (usually secondary to preexisting MDS)

AML, therapy-related

AML, other

Acute leukemias of ambiguous lineage (both myeloid & lymphoid phenotype)

History

marrow failure: anemia, infection, bleeding

- DIC occurs in acute promyelocytic leukemia

infiltration: hepato- & splenomegaly, gum hypertrophy, skin involvement

Acute promyelocytic leukemia (APL) differs from AML in that most patients present with coagulopathy. The coagulopathy has been described as DIC with associated hyperfibrinolysis. Acute promyelocytic leukemia (APL) has been associated with low levels of plasminogen, alpha2-plasmin inhibitor, and plasminogen activator inhibitor 1 found in fibrinolytic states. There is increased expression of annexin II, a receptor for plasminogen and plasminogen-activating factor, on the surface of leukemic promyelocytes.[7] This leads to overproduction of plasmin and fibrinolysis.

emedicine.medscape (http://emedicine.medscape.com/article/1495306-clinical)

Investigation results

*Dx now based on immunophenotyping & molecular methods - cytogenetic analysis affects Rx recommendations & guides prognosis

WCC often elevated but can be low or normal

blast cells may be few in peripheral blood (-> Dx on bone marrow biopsy)

auer rods

Treatment

supportive care

- walking can relieve fatigue

- bloods, platelets, IV fluids given as necessary

- G-CSF between chemo cycles

- neutropenic regimen

chemotherapy

- daunorubicin, cytarabine

- ~5 cycles given in 1 week blocks to achieve remission

bone marrow transplant

1. allogeneic

^from HLA-matched siblings / unrelated donor

^indicated during 1st remission in disease w poor prognosis

^use cyclophosphamide+total body irradiation to destroy leukemic cells & immune system before transplant

^ciclosporin+methotrexate may be used to reduce graft vs host disease

^^complications: GVDH, opportunistic infections, relapse of leukemia, infertility

^^prognosis: lower relapse rates, ~60% LT survivors, ~10% mortality

2. autologus

^used in intermediate prognosis disease

^autologus mobilised peripheral blood stem cell transplant may offer faster hematopoietic recovery & less morbidity

ALL

APML