Leukaemia acute C95.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 13.11.2021

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Synonym(s)

AL

Definition
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Clonal diseases of precursor cells of myelopoesis or lymphopoesis, characterized by an increase of blasts in bone marrow, blood and possibly also in other organs with displacement of normal blood formation.

Classification
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Acute leukemia is classified using cytological, cytochemical and immunological methods. 3 major groups can be distinguished:

Occurrence/Epidemiology
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AML: incidence: 2-3/100,000 population/year; much higher in older age (> 15/100,000 population/year).

ALL: incidence: approx. 1/100,000 population/year; in childhood < 5 LY (peak incidence): 5.3/100,000 population/year; beyond the 8th decade (peak incidence): 2.3/100,000 population/year.

Etiopathogenesis
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Often the cause is unknown. Increasingly, AML is described as occurring after chemotherapy (alkylants, topoisomerase II inhibitors) and radiation therapy of other malignant diseases (secondary leukemia - mostly myeloid leukemias). Patients with chromosomal abnormalities such as Klinefelter syndrome and Down syndrome (10-fold increased incidence) are genetically predisposed.

Chromosomal defects are found in almost all acute leukemias.

In AML, deletions on chromosomes 5 and 7, trisomy 8, and complex chromosomal aberrations, among others, have been described and are poor prognostic factors. Translocations t(8/21) and t(15/17) and aberrations at chromosome 16 have a rather favorable prognosis.

In ALL, unfavorable prognosis exists for patients with a Philadelphia translocation t(9/22) or even patients with t(4/11).

Triggering of ALL by retroviruses (HTLV1) is described endemically in some areas of Africa, Japan and the Caribbean.

Manifestation
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Appearance in all age groups. > 90% of acute childhood leukemias are acute lymphatic leukemias (ALL). Acute myelogenous leukaemias (AML) with their subtypes are more frequent in adulthood (age median around the age of 60). About 50% of all patients with AML are > 60 years old.

Clinical features
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Initial symptomatology (often characterized by hematopoietic insufficiency):
  • Anemia (paleness, loss of performance)
  • Thrombopoenia (petechial hemorrhages, increased bleeding of the gums)
  • Granulocytopenia (highly febrile infections, especially tonsillitis, inflammation of the gums and pneumonia in the foreground).
  • Moderate hepatosplenomegaly and lymph node enlargement (rather rare in acute myeloid leukemias).
  • Detection of possible specific infiltrates on the skin (see below leukemias of the skin), mucous membranes and ocular fundus.

Laboratory
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Blood count with reticulocytes and differential blood count, coagulation tests, blood group. If necessary FISH (fluorescence in situ hybridization) analysis of chromosomal aberrations. Bacteriological and virological analyses (signs of infection).

Diagnosis
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Diagnostically decisive is the finding of the bone marrow aspiration. In addition to light microscopic, cytological and cytochemical analysis, immunological, cytogenetic and molecular biological investigations are necessary.

Lumbar puncture if CNS involvement is suspected.

X-ray examination of the thorax or thoracic computer tomography (pneumonic infiltrates or mediastinal tumors, especially in T-ALL).

Abdominal sonography, renal sonography (if creatinine is elevated, think of renal involvement).

Sonographic examination of the lymph nodes, liver, spleen, testicles and CNS.

Literature
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  1. Faderl S et al (2003) The biology and therapy of adult acute lymphoblastic leukemia. Cancer 98: 1337-1354
  2. Rathnasabapathy R et al (2003) Management of acute myelogenous leukemia in the elderly. Cancer Control 10: 469-477
  3. Ravandi F et al (2004) New agents in acute myeloid leukemia and other myeloid disorders. Cancer 100: 441-454
  4. Thomas X et al (2003) Prognostic factors in adult acute lymphoblastic leukemia. Hematology 8: 233-242

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 13.11.2021