Last reviewed: November 2017
Last updated: February  2017

Introduction

Condition
Description

A malignant clonal disease that develops when a B/T-precursor-stage lymphoid progenitor cell becomes genetically altered through somatic changes and undergoes uncontrolled proliferation. Acute lymphocytic leukemia (ALL) is the most common leukemia in pediatrics, accounting for up to 80% of leukemias in this group and 20% of leukemias in adults. [1] Clinical presentation is heterogenous and reflects biological subtype. Most cases present with signs and symptoms associated with cytopenias. Enlarged lymph nodes are also frequently the initial cause for seeking medical attention by the patient. [2] T-lineage ALL is characterized by an older age of onset, male gender preponderance, and inferior outcome in comparison with B-ALL. [1] The cytogenetic abnormality has prognostic implications.

A cancer of B lymphocytes. Failure of B lymphocytes to undergo maturation and full differentiation leads to a monoclonal population of dysfunctional but self-renewing B lymphocytes. These lymphocytes can infiltrate lymphatic tissues and hematopoietic organs such as the liver, spleen, and bone marrow. A key risk factor is age over 60 years. Lymphadenopathy, splenomegaly (in 50% of cases), and shortness of breath and fatigue are key diagnostic factors. [3] [4] [5] It is diagnosed by CBC with differential, blood smear showing smudge cells, and flow cytometry. [3] [4] [5] Most cases are diagnosed on a routine CBC for an unrelated reason.

The clonal expansion of myeloid blasts in the bone marrow, peripheral blood or extramedullary tissues. Occurs predominantly in older adults. [6] Common findings are pallor, ecchymoses and petechiae. Acute promyelocytic leukemia (APML) is a form of acute myelogenous leukemia with distinct cytologic and clinical features, which has specific management. [7]

A malignant clonal disorder of the hematopoietic stem cell that results in marked myeloid hyperplasia of the bone marrow. [8] May also be called chronic granulocytic leukemia, chronic myelocytic leukemia, or chronic myeloid leukemia. Median age at presentation is around 53 years, and the only known risk is exposure to ionizing radiation. [9] [10] Many cases are asymptomatic but possible symptoms include fever, chills, malaise, weight loss, and night sweats. Around 75% of patients have splenomegaly; [11] all patients will have elevated WBC count. Diagnosis should be confirmed by the presence of the Philadelphia chromosome and/or the BCR-ABL rearrangement in peripheral blood or bone marrow cells. [12]

Blast crisis refers to the transformation of chronic myelogenous leukemia (CML) from the chronic or accelerated phase to the blast phase. Anemia, infections, abnormal bleeding, bone pain, and constitutional symptoms (night sweats, weight loss, fever) are common presenting complaints of blast-phase CML. The goal is to achieve a complete hematologic remission or at least return to the chronic phase in order to perform stem cell transplant.

A B-cell malignancy characterized commonly by symptoms of fatigue, a markedly enlarged spleen, and a distinctive histologic appearance on peripheral blood smear and bone marrow biopsy. Hairy cell leukemia (HCL) is characterized by B-cells with delicate cytoplasmic projections resembling hair. There is marked geographic variation in the frequency of HCL (also known as leukemic reticuloendotheliosis) and it is relatively uncommon. The median age of patients is 50 years and HCL affects more men than women. [13] It often presents with abdominal discomfort or fullness. This is attributed to splenomegaly, which is present in around 60% to 90% of patients. [13] [14] The disease is not curable. However, it is highly responsive to therapy and may be managed successfully for a decade or more.

Leukemias may cause pancytopenia through decreased production, or increased destruction or sequestration, of blood cells.

Neutropenia contributes to some of the typical symptoms of leukemia, including recurrent infections.

Contributors

Authors

BMJ Publishing Group

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This overview has been compiled using the information in existing sub-topics.

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