praktek Essentials

Akut lymphoblastic leukemia (ALL) adalah (klonal) penyakit ganas dari sumsum
tulang di mana awal prekursor limfoid berkembang biak dan menggantikan sel-sel
hematopoietik normal sumsum. SEMUA adalah jenis yang paling umum dari kanker
dan leukemia pada anak-anak di Amerika Serikat. Gambar di bawah menunjukkan
prekursor sel B ALL.

pemeriksaan diagnostik pasien dengan pre-B-sel leukemia limfoblastik akut. Aspirasi

sumsum tulang mengungkapkan Perancis-Amerika-Inggris L2 morfologi.

Lihat Childhood Akut Limfoblastik Leukemia: Diagnosis, Manajemen, dan

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Tanda dan gejala

Tanda dan gejala SEMUA meliputi berikut ini:

Demam
jumlah neutrofil menurun
Signs and symptoms of anemia, such as pallor, fatigue, dizziness, palpitations,
cardiac flow murmur, and dyspnea with even mild exertion
Bleeding (eg, from thrombocytopenia due to marrow replacement)
Disseminated intravascular coagulation (DIC) at diagnosis (about 10% of
cases)
Palpable lymphadenopathy
 Symptoms related to a large mediastinal mass (eg, shortness of breath),
particularly with T-cell ALL
Bone pain (severe and often atypical)
Left upper quadrant fullness and early satiety due to splenomegaly (about 10-
20% of cases)
Symptoms of leukostasis (eg, respiratory distress, altered mental status)
Renal failure in patients with a high tumor burden
Infections, including pneumonia
Petechiae (particularly on lower extremities) and ecchymoses
Signs relating to organ infiltration with leukemic cells and lymphadenopathy
Rashes from skin infiltration with leukemic cells

Diagnosis
Laboratory tests and other studies used in the workup for ALL include the following:

Complete blood count with differential

Coagulation studies
Peripheral blood smear
Chemistry profile, including lactic dehydrogenase, uric acid, liver function
studies, and BUN/creatinine
Appropriate cultures (in particular, blood cultures) in patients with fever or
other signs of infection
Chest x-ray
Computed tomography
Multiple-gated acquisition scanning
Electrocardiography
Bone marrow aspiration and biopsy (definitive for confirming leukemia)
Immunohistochemistry
Flow cytometry
Cytogenetics
Polymerase chain reaction
Gene expression profiling
Management
Treatment of ALL may include the following:

Induction chemotherapy (eg, standard 4- or 5-drug regimen, ALL-2, or hyper-

CVAD)
Consolidation chemotherapy
Maintenance chemotherapy
Intrathecal chemotherapy for central nervous system (CNS) prophylaxis
Supportive care (eg, blood products, antibiotics, growth factors)

Pathophysiology
The malignant cells of acute lymphoblastic leukemia (ALL) are lymphoid precursor
cells (ie, lymphoblasts) that are arrested in an early stage of development. This arrest
is caused by an abnormal expression of genes, often as a result of chromosomal
translocations. The lymphoblasts replace the normal marrow elements, resulting in a
marked decrease in the production of normal blood cells. Consequently,anemia,
thrombocytopenia, and neutropenia occur to varying degrees. The lymphoblasts also
proliferate in organs other than the marrow, particularly the liver, spleen, and lymph
nodes.

Etiology
Less is known about the etiology of acute lymphoblastic leukemia (ALL) in adults
compared with acute myelogenous leukemia (AML). Most adults with ALL have no
identifiable risk factors.

Although most leukemias occurring after exposure to radiation are AML rather than
ALL, an increased prevalence of ALL was noted in survivors of the Hiroshima
atomic bomb but not in those who survived the Nagasaki atomic bomb.

Rare patients have an antecedent hematologic disorder (AHD) such as

myelodysplastic syndrome (MDS) that evolves to ALL. However, most patients with
MDS that evolves to acute leukemia develop AML rather than ALL. Similarly, a
small number of patients receiving lenalidomide as maintenance therapy for multiple
myeloma have developed secondary ALL. [1]

Increasingly, cases of ALL with abnormalities of chromosome band 11q23 following

treatment with topoisomerase II inhibitors for another malignancy have been
described. However, most patients who develop secondary acute leukemia after
chemotherapy for another cancer develop AML rather than ALL.

Epidemiology
Acute lymphoblastic leukemia (ALL) is the most common type of cancer and
leukemia in children in the United States. ALL accounts for 75% of pediatric
leukemia cases. [2]

In adults, this disease is less common than acute myelogenous leukemia (AML).
Approximately 1000 new cases of ALL occur in adults each year. However, due to
the fact that there are more adults than children, the number of cases seen in adults is
comparable to that seen in children. ALL is slightly more common in males than in
females.

Di seluruh dunia, insiden tertinggi SEMUA terjadi di Italia, Amerika Serikat, Swiss,
dan Kosta Rika.

Prognosa
Hanya 20-40% dari orang dewasa dengan leukemia limfoblastik akut (ALL) yang
disembuhkan dengan pengobatan terkini.

Pasien dengan SEMUA dibagi menjadi tiga kelompok prognostik: risiko yang baik,
risiko menengah, dan risiko miskin.

kriteria risiko yang baik adalah sebagai berikut:

Tidak ada Sitogenetika merugikan

Usia lebih muda dari 30 tahun
sel darah (WBC) count putih kurang dari 30.000 / uL
remisi lengkap dalam waktu 4 minggu
risiko menengah termasuk orang-orang yang kondisinya tidak memenuhi kriteria baik
untuk risiko yang baik atau risiko miskin.