Acute erythroid leukemia (or "acute Di Guglielmo syndrome") is a rare form of acute myeloid leukemia

Acute myeloid leukemia

Acute myeloid leukemia , also known as acute myelogenous leukemia, is a cancer of the myeloid line of blood cells, characterized by the rapid growth of abnormal white blood cells that accumulate in the bone marrow and interfere with the production of normal blood cells. AML is the most common acute...

 where the myeloproliferation is of erythroblastic precursors.

It is defined at type "M6" under the FAB classification.

Types

Acute erythroid leukemias can be classified as follows:

• M6a; Erythroleukemia: Both Erythroid/Myeloid neoplastic proliferation

• M6b; Pure erythroid leukemia

M6a (Erythroleukemia)

50% or more of all nucleated bone marrow cells are erythroblasts, Dyserythropoiesis is prominent and 30% or more of the remaining cells (non- erythroid) are myeloblasts.

M6b (Pure erythroid leukemia)

In rare cases the erythroid lineage is the only obvious component of an acute leukemia

Leukemia

Leukemia or leukaemia is a type of cancer of the blood or bone marrow characterized by an abnormal increase of immature white blood cells called "blasts". Leukemia is a broad term covering a spectrum of diseases...

; a myeloblast

Myeloblast

The myeloblast is a unipotent stem cell, which will differentiate into one of the actors of the Granulocyte series.- Origin :These cells descend from the primitive reticulum cells, which are found in the stroma of the marrow. There is also an intermediate phase between the myeloblast and these...

 component is not apparent. The erythroid component consists predominantly or exclusively of proerythroblasts and early basophilic erythroblasts. These cells may constitute 90% or more of the marrow elements. Despite this lack of myeloblasts, these cases should be considered acute leukemias. In a WHO proposal the blastic leukemias that are limited to the erythroid series are designated pure erythroid malignancies.

M6c (Erythroleukemia and Pure erythroid leukemia)

Myeloblast- and proerythroblast-rich mixed variant.

Treatment

Treatment for erythroleukemia consists of chemotherapy

Chemotherapy

Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....

, frequently consisting of
cytarabine

Cytarabine

Cytarabine, or cytosine arabinoside, is a chemotherapy agent used mainly in the treatment of cancers of white blood cells such as acute myeloid leukemia and non-Hodgkin lymphoma. It is also known as Ara-C...

, daunorubicin

Daunorubicin

Daunorubicin or daunomycin is chemotherapeutic of the anthracycline family that is given as a treatment for some types of cancer. It is most commonly used to treat specific types of leukaemia...

, and idarubicin

Idarubicin

Idarubicin or 4-demethoxydaunorubicin is an anthracycline antileukemic drug. It inserts itself into DNA and prevents DNA from unwinding by interfering with the enzyme topoisomerase II. It is an analog of daunorubicin, but the absence of a methoxy group increases its fat solubility and cellular...

.

Prognosis

Acute erythroid leukemia (M6) has a relatively poor prognosis, with median survival for erythroleukemia patients of 36 weeks. The 36 week prognosis is due to the rareness of M6b. When looked at separately, prognosis comes back a little differently: M6B (3 ± 3.6 months) versus M6A (25 ± 28 months), and M6C (10 ± 13 months).

In pregnancy

Leukemia is rarely associated with pregnancy, affecting only about 1 in 10,000 pregnant women. How it is handled depends primarily on the type of leukemia. Acute leukemias normally require prompt, aggressive treatment, despite significant risks of pregnancy loss and birth defects, especially if chemotherapy is given during the developmentally sensitive first trimester.

External links

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