Thursday, March 8, 2012

Promyelocytic Leukemia

Promyelocytic Leukemia

Promyelocytic Leukemia a growth of the bone marrow in which there is a deficit of mature blood cells in the myeloid line of cells and a surplus of immature cells called promyelocytes. Promyelocytic leukemia is due to a translocation between chromosomes 15 and 17 which is symbolized t(15;17).

This translocation is not a mere indicator of promyelocytic leukemia but the main cause. Promyelocytic leukemia generally comes under the acute form leukemia. It is also termed as acute promyelocytic leukemia (APL).

In 1957, promyelocytic leukemia was first accepted as an individual disease entity. It accounts for 5-10% of cases of acute myeloid leukemia (AML). The peak incidence of promyelocytic leukemia is amongst young adults. Promyelocytic leukemia is thought of as a type of AML and is classified as the M3 variant of AML.

Symptoms of promyelocytic leukemia are generally nonspecific and comprise of fatigue, minor infections, or hemorrhagic diathesis. There is usually pancytopenia with anemia, low levels of the granulocytes and monocytes, and low levels of platelets. Transfusion is thus an alternative that can be availed of.

Treatment of promyelocytic leukemia is different from that for all other forms of AML. Majority patients are now treated with all-trans-retinoic acid (ATRA). ATRA is a form of "differentiation therapy." It activates the retinoid receptor RAR and causes the promyeloctes to mature and this deters them from proliferating.

Even though ATRA cannot eliminate the leukemic clone it can stimulate a complete diminution in most patients suffering from promyelocytic leukemia by causing the promyelocytic leukemia -blasts to mature. ATRA is therefore used in combination with chemotherapy including an anthracycline drug.

Chances of survival are better with the combination of ATRA and chemotherapy than chemotherapy alone. This is because ATRA combined with chemotherapy accounts for a slightly higher rate of complete remissions while allowing significantly fewer relapses.

Maintenance cure with ATRA, and possibly with low-dose chemotherapy, further reduces the occurrence of relapse. The advent of ATRA therapy has revolutionized the treatment of promyelocytic leukemia and clearly enhanced the prognosis.

Promyelocytic leukemia is connected with a characteristic cellular picture classified as M3 in the French-American-British Classification and responds positively to treatments including retinoids, chemotherapy and, most recently, arsenicals.

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