Acute erythrocyte leukemia is a rare form of acute myeloid leukemia (less than 5% of AML cases) where the

myeloproliferation Myeloproliferative neoplasms (MPNs) are a group of rare blood cancers in which excess red blood cells, white blood cells or platelets are produced in the bone marrow. ''Myelo'' refers to the bone marrow, ''proliferative'' describes the rapid growt ...

is of erythrocytic precursors. It is defined as type "M6" under the FAB classification.

Signs and symptoms

The most common symptoms of AEL are related to

pancytopenia Pancytopenia is a medical condition in which there is significant reduction in the number of almost all blood cells (red blood cells, white blood cells, platelets, monocytes, lymphocytes, etc.). If only two parameters from the complete blood coun ...

(a shortage of all types of blood cells), including

fatigue Fatigue describes a state of tiredness that does not resolve with rest or sleep. In general usage, fatigue is synonymous with extreme tiredness or exhaustion that normally follows prolonged physical or mental activity. When it does not resolve ...

infection An infection is the invasion of tissues by pathogens, their multiplication, and the reaction of host tissues to the infectious agent and the toxins they produce. An infectious disease, also known as a transmissible disease or communicable dise ...

s, and

mucocutaneous A mucocutaneous junction, or mucocutaneous boundary, is a region of the body in which mucosa transitions to skin. Mucocutaneous zones occur in animals, at the body orifices. In humans, mucocutaneous junctions are found at the lips, nostrils, conjun ...

bleeding. Almost half of people with AEL exhibit

weight loss Weight loss, in the context of medicine, health, or physical fitness, refers to a reduction of the total body mass, by a mean loss of fluid, body fat (adipose tissue), or lean mass (namely bone mineral deposits, muscle, tendon, and other conn ...

fever Fever, also referred to as pyrexia, is defined as having a body temperature, temperature above the human body temperature, normal range due to an increase in the body's temperature Human body temperature#Fever, set point. There is not a single ...

and night sweats at the time of diagnosis. Almost all people with AEL are

anemic Anemia or anaemia (British English) is a blood disorder in which the blood has a reduced ability to carry oxygen due to a lower than normal number of red blood cells, or a reduction in the amount of hemoglobin. When anemia comes on slowly, th ...

, and 77% have a

hemoglobin Hemoglobin (haemoglobin BrE) (from the Greek word αἷμα, ''haîma'' 'blood' + Latin ''globus'' 'ball, sphere' + ''-in'') (), abbreviated Hb or Hgb, is the iron-containing oxygen-transport metalloprotein present in red blood cells (erythrocyte ...

level under 10.0 g/dl. Signs of

thrombocytopenia Thrombocytopenia is a condition characterized by abnormally low levels of platelets, also known as thrombocytes, in the blood. It is the most common coagulation disorder among intensive care patients and is seen in a fifth of medical patients an ...

are found in about half of people with AEL.

Causes

The causes of AEL are unknown. Prior to a 2008 reclassification by the

World Health Organization The World Health Organization (WHO) is a specialized agency of the United Nations responsible for international public health. The WHO Constitution states its main objective as "the attainment by all peoples of the highest possible level of h ...

, cases that evolved from myelodysplastic syndromes, myeloproliferative neoplasms,

chemotherapy Chemotherapy (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs (chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemotherap ...

for other cancers or exposure to toxins were defined as ''secondary AEL''. These cases are now likely to instead be classified as '' acute myeloid leukemia with myelodysplasia-related changes'' or ''therapy-related AML''. +

Diagnosis

Acute erythroid leukemias can be classified as follows:

M6a (Erythroleukemia)

50% or more of all nucleated bone marrow cells are

erythroblast A nucleated red blood cell (NRBC), also known by several other names, is a red blood cell that contains a cell nucleus. Almost all vertebrate organisms have hemoglobin-containing cells in their blood, and with the exception of mammals, all of the ...

s, dyserythropoiesis is prominent and 20% or more of the remaining cells (non- erythroid) are

myeloblast The myeloblast is a unipotent stem cell which differentiates into the effectors of the granulocyte series. It is found in the bone marrow. Stimulation of myeloblasts by G-CSF and other cytokines triggers maturation, differentiation, proliferation a ...

M6b (Pure erythroid leukemia)

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

leukemia Leukemia ( also spelled leukaemia and pronounced ) is a group of blood cancers that usually begin in the bone marrow and result in high numbers of abnormal blood cells. These blood cells are not fully developed and are called ''blasts'' or ' ...

; a myeloblast component is not apparent. The erythroid component consists predominantly or exclusively of

proerythroblast A proerythroblast (or rubriblast, or pronormoblast) is the earliest of four stages in development of the normoblast. In histology, it is very difficult to distinguish it from the other "-blast" cells (lymphoblast, myeloblast, monoblast, and mega ...

s 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 generally follows that for other types of AML, not otherwise specified. It consists of

, frequently consisting of

cytarabine Cytarabine, also known as cytosine arabinoside (ara-C), is a chemotherapy medication used to treat acute myeloid leukemia (AML), acute lymphocytic leukemia (ALL), chronic myelogenous leukemia (CML), and non-Hodgkin's lymphoma. It is given by in ...

, daunorubicin, and idarubicin. It can also involve bone marrow transplantation.

Prognosis

Information on prognosis is limited by the rarity of the condition. Prognosis appears to be no different from AML in general, taking into account other risk factors. Acute erythroid leukemia (M6) has a relatively poor prognosis. A 2010 study of 124 patients found a

median In statistics and probability theory, the median is the value separating the higher half from the lower half of a data sample, a population, or a probability distribution. For a data set, it may be thought of as "the middle" value. The basic fe ...

overall survival of 8 months. A 2009 study on 91 patients found a median overall survival for erythroleukemia patients of 36 weeks, with no statistically significant difference to other AML patients. AEL patients did have a significantly shorter disease-free survival period, a median of 32 weeks, but this effect was explained by other prognostic factors. That is, AEL is often associated with other risk factors, like monosomal karyotypes and a history of myelodysplastic syndrome. 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.

Epidemiology

Acute erythroid leukemia is rare, accounting for only 3–5% of all acute myeloid leukemia cases. One study estimated an occurrence rate of 0.077 cases per 100,000 people each year. 64–70% of people with this condition are male, and most are elderly, with a median age of 65.

History

The first known case of acute erythroid leukemia was described in 1912 by M. Copelli under the name ''erythromatosis''. In 1917, Italian

hematologist Hematology (American and British English spelling differences#ae and oe, always spelled haematology in British English) is the branch of medicine concerned with the study of the cause, prognosis, treatment, and prevention of diseases related to ...

Giovanni Di Guglielmo Giovanni Di Guglielmo (22 September 1886 – 19 February 1961) was a Brazilian-born Italian hematologist, best known for the discovery of acute erythroid leukemia. Life and career Di Guglielmo was born in São Paulo, the son of Italian immigran ...

(1886–1962), expanded on the description, coining the name "eritroleucemia" (Italian for ''erythroleukemia''). Di Guglielmo was the first to recognize the leukemic nature of the condition, and it is sometimes referred to as Di Guglielmo's syndrome in recognition of his work. Ernst Neumark was widely credited for introducing Di Guglielmo's syndrome to English pathology. Chris Squire, bassist from the progressive rock group

Yes Yes or YES may refer to: * An affirmative particle in the English language; see yes and no Education * YES Prep Public Schools, Houston, Texas, US * YES (Your Extraordinary Saturday), a learning program from the Minnesota Institute for Talente ...

, died from complications related to acute erythroid leukemia on June 27, 2015.

References

External links

*
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{{Myeloid malignancy, us=y Acute myeloid leukemia