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Acute Erythroleukemia

AML-M6


Presentation

  • The karyotype was diploid in 29% of patients, and trisomy 8 was present in 8.3%. No patient with AML-M6 presented with good-risk cytogenetics. Also, 56 patients (52%) presented with a complex karyotype.[ncbi.nlm.nih.gov]
  • In terms of its clinical presentation, most patients have cytopenia(s) (often pancytopenia) rather than leukocytosis and splenomegaly.[academic.oup.com]
  • Di Guglielmo's ideas became better known internationally after his presentation on this subject at the Sixth Congress of the International Society of Haematology in Boston in 1956.[onlinelibrary.wiley.com]
  • Neutrophils present in the field exhibit weak reactivity and the control (not shown) was strongly positive.[archivesofpathology.org]
  • In recent years (2008 until the present), virtually all new AML cases at MDACC have been tested for NPM1, FLT3, RAS, and KIT gene mutations as part of the routine work-up.[journals.plos.org]
Falling
  • According to the WHO 2008 classification, erythroid-predominant myeloid neoplasms can fall into several categories based on the relative proportions of blasts and erythroid elements: MDS (ranging from low-grade entities such as RCUD and RCMD to RAEB2)[academic.oup.com]
  • In some cases of pure erythroid leukemia, the blasts may be intermediate in size or small, falling into the size range of ALL and malignant lymphoma.[archivesofpathology.org]
Fatigue
  • Signs and symptoms [ edit ] The most common symptoms of AEL are related to pancytopenia (a shortage of all types of blood cells), including fatigue, infections, and mucocutaneous bleeding. [2] Almost half of people with AEL exhibit weight loss, fever[en.wikipedia.org]
Easy Bruising
  • Symptoms of AML include: Fever Shortness of breath Easy bruising or bleeding Bleeding under the skin Weakness or feeling tired Weight loss or loss of appetite Tests that examine the blood and bone marrow diagnose AML.[fpnotebook.com]
Aspiration
  • A prerequisite is to perform an accurate count of at least 500 nucleated cells in well-prepared BM aspirate smears.[academic.oup.com]
  • A relapse was defined by more than 5% blasts in a bone marrow aspirate unrelated to bone marrow recovery or by the presence of extramedullary disease.[ncbi.nlm.nih.gov]
  • The bone marrow aspirate clot and biopsy specimens are usually hypercellular in both subtypes of AEL.[archivesofpathology.org]
  • Bone marrow aspirate smears showed 74% erythroid precursors with dysplastic features and 10% myeloblasts, representing 38% of all non-erythroid elements. The patient had a normal karyotype and no other genes tested were mutated.[journals.plos.org]
Confusion
  • However, these diseases were rare, and there was confusion regarding criteria for diagnosis.[academic.oup.com]
  • The basophilic cytoplasm of erythroblasts and neoplastic plasma cells also can be confused morphologically.37 Immunophenotypic analysis is useful for this differential diagnosis, as plasma cells express CD38, CD138, and cytoplasmic immunoglobulin, unlike[archivesofpathology.org]

Workup

  • Immunohistochemistry has a role in the workup of cases of AEL, particularly in cases in which the aspirate material is scant. Here we mention markers or applications not already addressed in the flow cytometry section above.[archivesofpathology.org]
Cytopenia
  • The differential diagnosis of AEL includes myeloid neoplasms that otherwise would be designated as RAEB type 1 (RAEB1) or even as refractory cytopenia with multilineage dysplasia (RCMD) or refractory cytopenia with unilineage dysplasia (RCUD).[academic.oup.com]
  • Fifty (46%) had MDS and/or cytopenias, with a median duration of 3.5 months (1-72) prior to the diagnosis of AML-M6.[ncbi.nlm.nih.gov]
  • Although a striking erythroblastemia can sometimes be identified in affected patients, examination of peripheral blood smears can be misleading because blood smears are often devoid of blasts and show only cytopenias (Figure 1).[archivesofpathology.org]

Treatment

  • Treatment Patients received different treatment regimens according to the period of diagnosis and prevailing studies. The treatment regimens were divided into: Group 1 – Regimens with Ara-C and Anthracyclines.[ncbi.nlm.nih.gov]
  • Erythroleukemia treatment at eMedicine a b Santos FP, Faderl S, Garcia-Manero G, et al. (December 2009). "Adult acute erythroleukemia: an analysis of 91 patients treated at a single institution".[en.wikipedia.org]
  • Possible risk factors include smoking, previous chemotherapy treatment, and exposure to radiation.[fpnotebook.com]
  • Treatment and prognostic impact of transient leukemia in neonates with Down syndrome. Blood. 2008 ; 111 : 2991 – 2998. 32. AML patients with Down syndrome have a high cure rate with AML-BFM therapy with reduced dose intensity.[academic.oup.com]
  • Common issues during treatment of patients with AEL include primary induction failure, relapse, and toxicity of chemotherapeutic agents.[archivesofpathology.org]

Prognosis

  • Some subtypes (M0, M7) have a worse prognosis 37 - 39, while others (M5) do not 40.[ncbi.nlm.nih.gov]
  • Prognosis appears to be no different to AML in general, taking into account other risk factors. [9] [10] Acute erythroid leukemia (M6) has a relatively poor prognosis.[en.wikipedia.org]
  • In contrast, the prognosis of patients with RAEB appeared to not be affected by the presence or absence of 50% erythroid precursors.[journals.plos.org]
  • The prognosis and cytogenetic characteristics of acute erythroid leukemia: a report of 55 cases [in Chinese]. Zhonghua Nei Ke Za Zhi 2006. 45 (9): 738 – 740. [Google Scholar][archivesofpathology.org]

Epidemiology

  • […] karyotypes and a history of myelodysplastic syndrome. [9] Prognosis is worse in elderly patients, those with a history of myelodysplastic syndrome, and in patients who had previously received chemotherapy for the treatment of a different neoplasm. [1] [11] Epidemiology[en.wikipedia.org]
  • Down’s syndrome and leukemia: epidemiology, genetics, cytogenetics and mechanisms of leukemogenesis. Cancer Genet Cytogenet. 1987 ; 28 : 55 – 76. 28. Risks of leukaemia and solid tumours in individuals with Down’s syndrome.[academic.oup.com]
Sex distribution
Age distribution

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