Lymphoid leukemia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Nima Nasiri, M.D.[2]

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Lymphoid leukemia
ICD-10 C91
ICD-9 204
MeSH D007945

Lymphoid leukemia Main Page

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Patient Information

Overview

Classification

Pathophysiology

Differentiating Lymphoid Lymphoma


Overview

Lymphoid leukemia is a monoclonal disorder which is a result of clonal proliferation and progressive accumulation of functionally incompetent lymphocytes in circulation, bone marrow, spleen, lymphoid tissues. Lymphocytic leukemia is more frequent than non-lymphocytic and other myeloproliferative diseases. The chronic form (CLL) affects older adult, onset is insidious. Acute lymphoblastic leukemia (ALL) is more common in children, peak incidence around 2 to 3 years of age. The identification of cytogenetic abnormalities is highly relevant for the prognosis of ALL. Patients may have findings associated with anemia, neutropenia, and/or thrombocytopenia due to bone marrow involvement. The white blood cell count may be decreased, normal, or markedly elevated. Symptoms can include fatigue, infections, or easy/spontaneous bruising or bleeding. Arthralgias and constitutional symptoms (eg, fever, night sweats, unintentional weight loss) are often present but are generally mild. Hepatomegaly, splenomegaly, and/or lymphadenopathy can be seen as well. Central nervous system (CNS) involvement may present as cranial neuropathies or meningeal symptoms. Lymphoblasts can have different surface molecules called cluster of differentiation (CD) which can be detected by flow cytometry.

Classification

ALL classification

There are two types of classifications for acute lymphoblastic leukemia : World Health Organization (WHO) and French-American-British (FAB)[1][2][3][4]

WHO classification of acute lymphoblastic leukemia

  • B lymphoblastic leukemia/lymphoma:
    • B lymphoblastic leukemia/lymphoma, Not otherwise specified
    • B lymphoblastic leukemia/lymphoma with recurrent genetic abnormalities:
    • B lymphoblastic leukemia/lymphoma with t(9;22),BCR-ABL[5]
    • B lymphoblastic leukemia/lymphoma t(11q23); MLL rearrangement[6]
    • B lymphoblastic leukemia/lymphoma with t(12;21)[7]
    • B lymphoblastic leukemia/lymphoma with hyperdiploidy
    • B lymphoblastic leukemia/lymphoma with hypodiploidy[8]
    • B lymphoblastic leukemia/lymphoma t(5;14)
    • B lymphoblastic leukemia/lymphoma t(1;19)
  • T lymphoblastic leukemia/lymphoma:

FAB classification of acute lymphoblastic leukemia (for historical purposes):[9]

CLL classification

  • There are two staging systems in order to classify CLL:

Pathophysiology

Physiology

Pathogenesis

  • It is understood that lymphoid leukemia is a result of overproduction of cells which is caused by either activation or inactivation of genes.[18][19][20][21]

Genetics

  • Activation or/and inactivation of genes plays an important role in the pathogenesis and prognosis of lymphoid leukemia.
  • Epigenetic and genetic alterations are two mechanisms in leukemia.
  • Abnormal methylation of DNA and histone modifications are important mechanisms in tumor suppressor silencing, contributing to leukemogenesis along with genetic alterations.
  • Epigenetic mechanisms are the most prevalent inactivation ones in lymphoid leukemia and involve the genes implicated in several cellular mechanisms, including gene expression and transcription, cell-cycle regulation and apoptosis.[23]

Associated Conditions

Conditions associated with lymphoid leukemia include:[24]

Differentiating Lymphoid Leukemia

Characteristics ALL CLL
Microscopy
ALL, hand-mirror cells. Source: wikimedia, transferred from wikipedia
CLL , Smudge cells. en.wikipedia Source: Mary Ann Thompson
Age of onset Children (age < 10 years old) Adult onset
Etiology Chromosomal aberration resulting in abnormal transcription factors that affect development of B and T cells Chromosomal deletion or possible somatic mutation of naive B cells
Morphology Scanty, basophilic cytoplasm sometimes with a single long projection (‘hand-mirror cell’), condensed chromatin, small nucleoli[25][26] Smudge cells, condensed chromatin, scant cytoplasm, small nucleoli[27]
Cell involved Immature B or T cells Peripheral B or T cells
Clinical presentation Stormy onset, symptoms related to depressed marrow function, bone pain, CNS manifestations. Asymptomatic or nonspecific, hepatosplenomegaly , lymphadenopathy
Demographic Most common leukemia in children Most common leukemia in adults, twice as common in men.
CBC result Anemia, thrombocytopenia, variable WBC's, and lymphoblast > 30% Lymphocytosis > 5000/ul , low platelets in 20-30%
CD markers CD19, CD79a, CD22 (cytoplasmic), CD24, CD10, PAX5, and TdT (terminal deoxyteransferase) CD5, CD19, CD20 (dim), CD 23, and an absence of FMC-7 staining[28]

References

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