Prolymphocytic leukemia

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Prolymphocytic leukemia Main page

Patient Information

B-cell prolymphocytic leukemia Patient Information
T-cell prolymphocytic leukemia Patient Information

Overview

Causes

Classification

B-cell prolymphocytic leukemia
B-cell prolymphocytic leukemia

Differentiating B-cell prolymphocytic leukemia from T-cell prolymphocytic leukemia

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Carlos A Lopez, M.D. [2], Maria Fernanda Villarreal, M.D. [3]

For more information regarding B-cell prolymphocytic leukemia, please click here.
For more information regarding T-cell prolymphocytic leukemia, please click here.

Overview

Prolymphocytic leukemia is a rare lymphoid leukemia, which account for only about 2% of all mature lymphoid leukemias. Prolymphocytic leukemias present similar to leukemia. Like lymphomas, they originate in the lymphocytes, but do not form solid tumors. Prolymphocytic leukemias are also considered lymphoproliferative disorders, which lymphocytes are produced in large amounts. Prolymphocytic leukemia may be classified according to the type of cell involved: B-cell prolymphocytic leukemia and T-cell prolymphocytic leukemia. Originally, it was thought to be a rare variation of chronic lymphocytic leukemia, but is now considered a distinct disease. It is usually classified as a kind of chronic lymphocytic leukemia. Although these 2 types of prolymphocytic leukemias share some of the same characteristics, the World Health Organization (WHO) classifies them as different types of lymphoid leukemias.[1]

Classification

For more details about each specific type of prolymphocytic leukemia, please select:

 
 
 
 
Prolymphocytic leukemia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
B-cell prolymphocytic leukemia
 
 
 
 
 
T-cell prolymphocytic leukemia

Differentiating B-Prolymphocytic Leukemia from T-Prolymphocytic Leukemia

In order to distinguish B-prolymphocytic leukemia from T-prolymphocytic leukemia, see the table below.

Characteristics B-cell Prolymphocytic Leukemia T-cell Prolymphocytic Leukemia
Epidemiology
  • Rare
  • Very rare
Age
  • 60-70 years
  • 60-70 years
Onset
Clinical Features

B symptoms

Diagnosis Peripheral blood smear
  • Prolymphocytes (90%)

Biomarkers

  • CD20+
Peripheral blood smear

Biomarkers

  • CD52+

Differentiating Prolymphocytic Leukemia from other Leukemias

Differential Diagnosis Surface Immunoglobulin CD5 CD22/FMC7 CD23 CD79b CD103
Prolymphocytic leukaemia Strongly positive Negative Positive Negative Positive Negative
Hairy cell leukaemia Strongly positive Negative Positive Negative Positive/Negative Positive
Chronic lymphocytic leukaemia Weakly positive Positive Negative Positive Negative Positive/Negative
Mantle cell lymphoma Positive Positive Strongly positive Negative Strongly positive Negative
Follicular lymphoma Strongly positive Negative Positive Negative Strongly positive Negative


Disease Etiology Clinical Manifestation Laboratory Findings Gold standard diagnosis Associated findings
Demography History Symptoms Signs Lab Histopathology
Acute myelogenous leukemia[2][3]
  • Clonal proliferation of malignant myeloid blast cells in the marrow
  • Genetic abnormalities t(8;21), inv(16), and t(15;17)
  • The most common leukemia in adults
  • Median age of 63 years old
Acute lymphoblastic leukemia[4][5]
  • Arrest of lymphoblasts
  • Chromosomal translocations: t(9;22) , t(12;21), t(5;14), t(1;19)
  • The most common cancer in children
  • Peak 2-5 years of age
  • Boys > girls
  • History of cancer
  • History of drug exposure
  • CNS involvement
Chronic myelogenous leukemia[6][7]
  • Median age 50 years old
Disease Etiology Demography History Symptoms Signs Lab Histopathology Gold standard diagnosis Associated findings
Chronic lymphocytic leukemia[8]
  • The most common leukemia in adults in western countries
  • M > F
  • Median age 70 years old
Hairy cell leukemia[9][10]
  • Accumulation of small mature B cell lymphoid cells with abundant cytoplasm and "hairy" projections
  • BRAF mutation
  • Uncommon
  • Median age 50 to 55 years old
  • M >> F
  • More common in Caucasians than Blacks
Large granular lymphocytic leukemia[11][12]
  • Clonal proliferation of cytotoxic T cells
  • Dysregulation of apoptosis through abnormalities in the Fas/Fas ligand pathway
  • Rare
  • Median age 60 years
  • M = F
  • Autoimmune diseases
  • Lymphoproliferative disorders
  • Mostly asymptomatic
  • Large lymphocytes with a condensed round or oval nucleus, abundant pale basophilic cytoplasm, and small azurophilic granules
  • Biopsy and flow cytometry + T-cell receptor gene rearrangement studies
  • Recurrent bacterial infection
Chronic neutrophilic leukemia[13]
  • Mature granulocytic proliferation in the blood and marrow
  • Point mutations in the CSF3R gene
  • Very rare
  • M = F
  • Peripheral blood neutrophilia (> 25 x 109/L) with myeloid precursors (promyelocytes, myelocytes, metamyelocytes)
  • Elevated leukocyte alkaline phosphatase
  • Toxic granulation in the neutrophils
  • Nuclear hypersegmentation
  • Increased myeloid:erythroid ratio > 20:1
  • WHO diagnostic criteria include leukocytosis of ≥ 25 x 109/L
  • More than 80% neutrophils,
  • Less than 10% circulating neutrophil precursors with blasts
  • Poor prognosis
  • Absence of the Philadelphia chromosome or a BCR/ABL fusion gene
Disease Etiology Demography History Symptoms Signs Lab Histopathology Gold standard diagnosis Associated findings
Chronic eosinophilic leukemia


Chronic monocytic leukemia
Prolymphocytic leukemia (PLL)
T-cell large granular lymphocytic leukemia (TLGL)
Disease Etiology Demography History Symptoms Signs Lab Histopathology Gold standard diagnosis Associated findings
Aggressive NK-cell leukemia (ANKL)
Adult T-cell leukemia/lymphoma (ATLL)
Sezary syndrome
Myelodysplastic syndrome Biopsy
  • Leukemia transformation
  • Acquired pseudo-Pelger-Huët anomaly
  • Infection
Myeloproliferative disorders
Leukemoid reaction

References

  1. "World Health Organization".
  2. Saif A, Kazmi S, Naseem R, Shah H, Butt MO (August 2018). "Acute Myeloid Leukemia: Is That All There Is?". Cureus. 10 (8): e3198. doi:10.7759/cureus.3198. PMID 30410824. Vancouver style error: initials (help)
  3. Estey EH (April 2013). "Acute myeloid leukemia: 2013 update on risk-stratification and management". Am. J. Hematol. 88 (4): 318–27. doi:10.1002/ajh.23404. PMID 23526416.
  4. Sawalha Y, Advani AS (March 2018). "Management of older adults with acute lymphoblastic leukemia: challenges & current approaches". Int J Hematol Oncol. 7 (1): IJH02. doi:10.2217/ijh-2017-0023. PMC 6176956. PMID 30302234.
  5. Portell CA, Advani AS (April 2014). "Novel targeted therapies in acute lymphoblastic leukemia". Leuk. Lymphoma. 55 (4): 737–48. doi:10.3109/10428194.2013.823493. PMID 23841506.
  6. Saußele S, Silver RT (April 2015). "Management of chronic myeloid leukemia in blast crisis". Ann. Hematol. 94 Suppl 2: S159–65. doi:10.1007/s00277-015-2324-0. PMID 25814082.
  7. Eden RE, Coviello JM. PMID 30285354. Missing or empty |title= (help)
  8. Rai KR, Jain P (March 2016). "Chronic lymphocytic leukemia (CLL)-Then and now". Am. J. Hematol. 91 (3): 330–40. doi:10.1002/ajh.24282. PMID 26690614.
  9. Troussard X, Cornet E (December 2017). "Hairy cell leukemia 2018: Update on diagnosis, risk-stratification, and treatment". Am. J. Hematol. 92 (12): 1382–1390. doi:10.1002/ajh.24936. PMC 5698705. PMID 29110361.
  10. Wierda WG, Byrd JC, Abramson JS, Bhat S, Bociek G, Brander D, Brown J, Chanan-Khan A, Coutre SE, Davis RS, Fletcher CD, Hill B, Kahl BS, Kamdar M, Kaplan LD, Khan N, Kipps TJ, Lancet J, Ma S, Malek S, Mosse C, Shadman M, Siddiqi T, Stephens D, Wagner N, Zelenetz AD, Dwyer MA, Sundar H (November 2017). "Hairy Cell Leukemia, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology". J Natl Compr Canc Netw. 15 (11): 1414–1427. doi:10.6004/jnccn.2017.0165. PMID 29118233.
  11. Matutes E (March 2017). "Large granular lymphocytic leukemia. Current diagnostic and therapeutic approaches and novel treatment options". Expert Rev Hematol. 10 (3): 251–258. doi:10.1080/17474086.2017.1284585. PMID 28128670.
  12. Oshimi K (2017). "Clinical Features, Pathogenesis, and Treatment of Large Granular Lymphocyte Leukemias". Intern. Med. 56 (14): 1759–1769. doi:10.2169/internalmedicine.56.8881. PMC 5548667. PMID 28717070.
  13. Elliott MA, Tefferi A (August 2018). "Chronic neutrophilic leukemia: 2018 update on diagnosis, molecular genetics and management". Am. J. Hematol. 93 (4): 578–587. doi:10.1002/ajh.24983. PMID 29512199.

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