Acute erythroid leukemia

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(Redirected from Di Guglielmo syndrome)


Acute erythroid leukemia
Multinucleated erythroblast in acute erythroid leukemia
Synonyms N/A
Pronounce N/A
Specialty N/A
Symptoms Anemia, fatigue, pallor, dyspnea, bleeding, infections
Complications Acute myeloid leukemia, bone marrow failure
Onset Typically in adulthood
Duration Chronic
Types Erythroleukemia, Pure erythroid leukemia
Causes Genetic mutations, radiation exposure, chemical exposure
Risks Previous chemotherapy, radiation therapy, genetic predisposition
Diagnosis Bone marrow biopsy, blood tests, cytogenetic analysis
Differential diagnosis Myelodysplastic syndrome, other types of leukemia
Prevention Avoidance of known risk factors
Treatment Chemotherapy, stem cell transplant
Medication N/A
Prognosis Variable, generally poor
Frequency Rare
Deaths N/A


Acute erythroid leukemia
Multinucleated erythroblast in acute erythroid leukemia

Acute erythroid leukemia is a rare subtype of acute myeloid leukemia (AML) characterized by a predominant proliferation of immature erythroid precursors. It is classified under the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia.

Classification

Acute erythroid leukemia is classified into two main subtypes:

  • Erythroleukemia (erythroid/myeloid type): This subtype is characterized by a significant proliferation of both erythroid and myeloid precursors. The bone marrow shows a predominance of erythroid precursors, with at least 50% of nucleated cells being erythroid and at least 20% of non-erythroid cells being myeloblasts.
  • Pure erythroid leukemia: This subtype is characterized by a proliferation of immature erythroid precursors without a significant myeloid component. The bone marrow is composed of more than 80% immature erythroid cells.

Pathophysiology

The pathophysiology of acute erythroid leukemia involves the clonal proliferation of erythroid precursors in the bone marrow. This results in ineffective erythropoiesis and anemia. The exact molecular mechanisms are not fully understood, but genetic mutations and chromosomal abnormalities are often involved.

Clinical Features

Patients with acute erythroid leukemia typically present with symptoms related to anemia, such as fatigue, pallor, and dyspnea. Other symptoms may include thrombocytopenia leading to bleeding tendencies and leukopenia resulting in increased susceptibility to infections.

Diagnosis

The diagnosis of acute erythroid leukemia is based on bone marrow examination. The bone marrow is hypercellular with a predominance of erythroid precursors. Cytogenetic and molecular genetic studies are often performed to identify specific genetic abnormalities.

Treatment

The treatment of acute erythroid leukemia is similar to other subtypes of acute myeloid leukemia and typically involves chemotherapy. The specific regimen may vary depending on the patient's age, overall health, and specific genetic mutations present.

Prognosis

The prognosis of acute erythroid leukemia is generally poor compared to other subtypes of AML. Factors influencing prognosis include age, performance status, and specific genetic abnormalities.

Related pages

Acute erythroid leukemia

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Contributors: Prab R. Tumpati, MD