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Leukemia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Seyedmahdi Pahlavani, M.D. [2], Usama Talib, BSc, MD [3], Sadaf Sharfaei M.D.[4]; Grammar Reviewer: Natalie Harpenau, B.S.[5]

Synonyms and keywords: Leukaemia

Overview

Overview

Leukemia (Greek leukos, “white”; haima, “blood”) can be defined as a group of hematopoietic stem cell malignancies due to genetic abnormalities that may lead to clonal proliferation of these cells. These group of diseases are classified based on the type of hematopoietic stem cell involved and the duration of the disease. The leukemias are the most common malignancies among children younger than 15 years. Among them, Acute Lymphoblastic Leukemia (ALL) is the most common leukemia and accounts for 77% of childhood leukemia. However, Chronic Lymphocytic Leukemia (CLL) is the most common form of leukemia in adults, and it accounts for 30% of all leukemias in the United States. The increased rate of proliferation and decreased rate of apoptosis in this progeny of cells may compromise normal bone marrow function and ultimately result in marrow failure. Clinical manifestations, diagnosis, laboratory findings, and therapy are different according to the type of malignancy.

Classification

Classification

Leukemia may be classified as follows:

 
 
 
 
 
 
 
 
Leukemia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Lymphoid progeny
 
 
 
 
 
 
 
Myeloid progeny
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Acute lymphoblastic leukemia (ALL)
 
 
 
Chronic lymphocytic leukemia (CLL)
 
Acute Myeloid Leukemia (AML)
 
 
 
Chronic myeloid leukemia (CML)




Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Classification

Leukemia is clinically and pathologically subdivided into several large groups. The first division is between its acute and chronic forms:

  • Acute leukemia is characterized by the rapid increase of immature blood cells. This crowding makes the bone marrow unable to produce healthy blood cells. Acute forms of leukemia can occur in children and young adults. In fact, it is a more common cause of death for children in the United States than any other type of malignant disease. Immediate treatment is required for acute leukemias due to the rapid progression and accumulation of the malignant cells, which then spill over into the bloodstream and spread to other organs of the body. Central nervous system (CNS) involvement is uncommon, but the disease can occasionally cause cranial nerve palsies.
  • Chronic leukemia is distinguished by the excessive build up of relatively mature, abnormal blood cells. Typically taking months or years to progress, the cells are produced at a much higher rate than normal cells, resulting in many abnormal white blood cells in the blood. Chronic leukemia mostly occurs in older people but can theoretically occur in any age group. While acute leukemia must be treated immediately, chronic forms are sometimes monitored for a some time before treatment to ensure maximum effectiveness of therapy.

Additionally, the diseases are subdivided according to which kind of blood cell is affected. This split divides leukemias into lymphoblastic or lymphocytic leukemias and myeloid or myelogenous leukemias:

Combining these two classifications provides a total of four main categories:

Four Major Kinds of Leukemia
Cell type Acute Chronic
Lymphocytic leukemia
(or “lymphoblastic”)		 Acute lymphoblastic leukemia (ALL) Chronic lymphocytic leukemia (CLL)
Myelogenous leukemia
(also “myeloid” or “nonlymphocytic”)		 Acute myelogenous leukemia (AML) Chronic myelogenous leukemia (CML)

Within these main categories, there are typically several subcategories. Finally, hairy cell leukemia is usually considered to be outside of this classification scheme.

  • Acute lymphoblastic leukemia (ALL) is the most common type of leukemia in young children. This disease also affects adults, especially those age 65 and older. Standard treatments involve either chemotherapy or radiation. The survival rates vary by age: 85% in children and 50% in adults.[1]
  • Chronic lymphocytic leukemia (CLL) most often affects adults over the age of 55. It sometimes occurs in younger adults, but it almost never affects children. Two-thirds of the affected individuals are men. The five-year survival rate is 75%.[2] It is incurable, but there are many effective treatments.
  • Acute myelogenous leukemia (AML) occurs more commonly in adults than in children, and more commonly in men than women. AML is treated with chemotherapy. The five-year survival rate is 40%.[3]
  • Chronic myelogenous leukemia (CML) occurs mainly in adults. A very small number of children also develop this disease. Treatment is with imatinib (Gleevec) or other drugs. The five-year survival rate is 90%.[4][5]
  • Hairy cell leukemia (HCL) is sometimes considered a subset of CLL but does not fit neatly into this pattern. About 80% of affected individuals are adult men. There are no reported cases in young children. HCL is incurable, but easily treatable. Survival is 96% to 100% at ten years.[6]

References

  1. ↑ Harrison’s Principles of Internal Medicine, 16th Edition, Chapter 97. Malignancies of Lymphoid Cells. Clinical Features, Treatment, and Prognosis of Specific Lymphoid Malignancies.
  2. ↑ Finding Cancer Statistics » Cancer Stat Fact Sheets »Chronic Lymphocytic Leukemia National Cancer Institute
  3. ↑ Colvin GA, Elfenbein GJ (2003). “The latest treatment advances for acute myelogenous leukemia”. Med Health R I. 86 (8): 243–6. PMID 14582219.
  4. ↑ Patients with Chronic Myelogenous Leukemia Continue to Do Well on Imatinib at 5-Year Follow-Up Medscape Medical News 2006
  5. ↑ Updated Results of Tyrosine Kinase Inhibitors in CML ASCO 2006 Conference Summaries
  6. ↑ Else M, Ruchlemer R, Osuji N; et al. (2005). “Long remissions in hairy cell leukemia with purine analogs: a report of 219 patients with a median follow-up of 12.5 years”. Cancer. 104 (11): 2442–8. doi:10.1002/cncr.21447. PMID 16245328.

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Differentiating Leukemia from other Diseases

Differentiating Leukemia from other Diseases

Leukemia must be differentiated from various diseases that cause weight loss, night sweats, hepatosplenomegaly, and palpable lymph nodes, such as hairy cell leukemia, prolymphocytic leukemia, follicular lymphoma, and mantle cell lymphoma. Based on the expression of cell surface markers, the table below differentiates different types of leukemia from other diseases that cause similar clinical presentations:[1]

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 bone 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]​
  • Rare
  • Median age 60 years
  • M = F
  • 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 bone marrow
  • Point mutations in the CSF3R gene
  • Very rare
  • M = F
  • 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
  • There is no known cause for chronic eosinophilic leukemia.
  • It hasn’t been linked to a specific chromosome or genetic abnormality.
  • Unknown
  • Unknown
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)[14][15][16][17][18][19]
  • Adult T-cell leukemia is caused by an infection with HTLV.
  • Common genetic mutations involved in the development of adult T-cell leukemia can be found here.
  • The incidence of adult T-cell leukemia increases with age, and the median age at diagnosis is 57 years.
  • Males are more commonly affected with adult T-cell leukemia than females.
  • The male to female ratio is approximately 1.4 to 1.
Sezary syndrome[20][21][22][23][24][25][26][27]
  • The cause of Sezary syndrome has not been identified.
  • Sezary syndrome might have one or more of the chromosomal abnormalities, such as the loss or gain of genetic.
  • The prevalence of Sezary syndrome is exact unknown.
  • The median age at diagnosis of Sézary syndrome is 60 years of age.
  • Sezary syndrome is more commonly observed among elderly patients.
  • Males are more commonly affected with Sezary syndrome than females(2:1).
Myelodysplastic syndrome Biopsy
  • Leukemia transformation
  • Acquired pseudo-Pelger-Huët anomaly
  • Infection
Myeloproliferative disorders
Leukemoid reaction
Epidemiology and Demographics

Epidemiology and Demographics

Prevalence

  • In the United States, the age-adjusted prevalence of leukemia is 75.3 per 100,000 in 2011.[29]

Incidence

  • The delay-adjusted incidence of leukemia in 2011 was estimated as 15.48 per 100,000 individuals in the United States.
  • In 2011, the age-adjusted incidence of leukemia was 13.66 per 100,000 individuals in the United States.

Age

  • The overall age-adjusted incidence of leukemia in the United States between 2007 and 2011 was 13 per 100,000 occurrences. The age-adjusted incidence of leukemia by age category is:
    • Under 65 years: 6.5 per 100,000
    • 65 and over: 57.9 per 100,000
  • Shown below is a table depicting the overall age-adjusted incidence of leukemia per 100,000 individuals by age in the United States between 2007 and 2011 for the different types of leukemia.
Acute lymphoblastic leukemia Chronic lymphocytic leukemia Acute myeloid leukemia Chronic myeloid leukemia
All ages 1.7 4.4 3.8 1.7
<65 1.7 1.4 1.8 0.9
≥65 1.6 25.2 17.5 6.8

Gender

  • In the United States, the age-adjusted prevalence of leukemia by gender in 2011 was:
    • In males: 92.7 per 100,000
    • In females: 60.7 per 100,000
  • In the United States, the delay-adjusted incidence of leukemia by gender in 2011 was:
    • In males: 19.93 per 100,000 persons
    • In females: 11.89 per 100,000 persons
  • In the United States, the age-adjusted incidence of leukemia by gender on 2011 was:
    • In males: 17.58 per 100,000 persons
    • In females: 10.49 per 100,000 persons
  • Shown below is an image depicting the delay-adjusted incidence and observed incidence of leukemia by gender and race in the United States between 1975 and 2011. These graphs were gathered from SEER: The Surveillance, Epidemiology, and End Results Program of the National Cancer Institute.
These graphs are adapted from SEER: The Surveillance, Epidemiology, and End Results Program of the National Cancer Institute. – Delay-adjusted incidence and observed incidence of leukemia by gender and race in the United States between 1975 and 2011

Race

  • Shown below is a table depicting the age-adjusted prevalence of leukemia by race in 2011 in the United States.
All Races White Black Asian/Pacific Islander Hispanic
Age-adjusted prevalence 75.3 per 100,000 83.5 per 100,000 45.9 per 100,000 41.2 per 100,000 57.1 per 100,000
  • Shown below is an image depicting the incidence of leukemia by race in the United States between 1975 and 2011.

Incidence of leukemia by race in the United States between 1975 and 2011

API: Asian/Pacific Islander; AI/AN: American Indian/ Alaska Native

Prognosis

Prognosis

5-Year Survival

  • Between 2004 and 2010, the 5-year relative survival of patients with leukemia was 60.3%.
  • When stratified by age, the 5-year relative survival of patients with leukemia was 68.5% (44.1% for patients <65 and ≥ 65 years of age respectively).
  • Shown below is a table depicting the 5-year relative survival of patients by the type of leukemia in the United States between 2004 and 2010.
Acute lymphoblastic leukemia Chronic lymphocytic leukemia Acute myeloid leukemia Chronic myeloid leukemia
5-year survival 70% 83.5% 25.4% 59.9%
References

References

  1. ↑ Hoffbrand V, Moss P. Essential Haematology. John Wiley & Sons; 2011
  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.
  14. ↑ Human T-lymphotropic virus. Wikipedia (2015) https://en.wikipedia.org/wiki/Human_T-lymphotropic_virus#Transmission Accessed on November, 3 2015
  15. ↑ Matutes E (2007). “Adult T-cell leukaemia/lymphoma”. J. Clin. Pathol. 60 (12): 1373–7. doi:10.1136/jcp.2007.052456. PMC 2095573. PMID 18042693.
  16. ↑ Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015
  17. ↑ Mahieux R, Gessain A (2007). “Adult T-cell leukemia/lymphoma and HTLV-1”. Curr Hematol Malig Rep. 2 (4): 257–64. doi:10.1007/s11899-007-0035-x. PMID 20425378.
  18. ↑ Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015
  19. ↑ Matutes E (2007). “Adult T-cell leukaemia/lymphoma”. J Clin Pathol. 60 (12): 1373–7. doi:10.1136/jcp.2007.052456. PMC 2095573. PMID 18042693.
  20. ↑ Wong HK, Mishra A, Hake T, Porcu P (October 2011). “Evolving insights in the pathogenesis and therapy of cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome)”. Br. J. Haematol. 155 (2): 150–66. doi:10.1111/j.1365-2141.2011.08852.x. PMC 4309373. PMID 21883142.
  21. ↑ Woollard WJ, Pullabhatla V, Lorenc A, Patel VM, Butler RM, Bayega A, Begum N, Bakr F, Dedhia K, Fisher J, Aguilar-Duran S, Flanagan C, Ghasemi AA, Hoffmann RM, Castillo-Mosquera N, Nuttall EA, Paul A, Roberts CA, Solomonidis EG, Tarrant R, Yoxall A, Beyers CZ, Ferreira S, Tosi I, Simpson MA, de Rinaldis E, Mitchell TJ, Whittaker SJ (June 2016). “Candidate driver genes involved in genome maintenance and DNA repair in Sézary syndrome”. Blood. 127 (26): 3387–97. doi:10.1182/blood-2016-02-699843. PMID 27121473.
  22. ↑ Wilcox RA (January 2016). “Cutaneous T-cell lymphoma: 2016 update on diagnosis, risk-stratification, and management”. Am. J. Hematol. 91 (1): 151–65. doi:10.1002/ajh.24233. PMC 4715621. PMID 26607183.
  23. ↑ Horesh N, Horowitz NA (October 2014). “Does gender matter in non-hodgkin lymphoma? Differences in epidemiology, clinical behavior, and therapy”. Rambam Maimonides Med J. 5 (4): e0038. doi:10.5041/RMMJ.10172. PMC 4222427. PMID 25386354.
  24. ↑ Al Hothali GI (June 2013). “Review of the treatment of mycosis fungoides and Sézary syndrome: A stage-based approach”. Int J Health Sci (Qassim). 7 (2): 220–39. PMC 3883611. PMID 24421750.
  25. ↑ Wilcox RA (January 2016). “Cutaneous T-cell lymphoma: 2016 update on diagnosis, risk-stratification, and management”. Am. J. Hematol. 91 (1): 151–65. doi:10.1002/ajh.24233. PMC 4715621. PMID 26607183.
  26. ↑ Yamashita T, Abbade LP, Marques ME, Marques SA (2012). “Mycosis fungoides and Sézary syndrome: clinical, histopathological and immunohistochemical review and update”. An Bras Dermatol. 87 (6): 817–28, quiz 829–30. PMC 3699909. PMID 23197199.
  27. ↑ “Extracorporeal photophoresis: an evidence-based analysis”. Ont Health Technol Assess Ser. 6 (6): 1–82. 2006. PMC 3379535. PMID 23074497.
  28. ↑ Olsen, Elise A. (2015). “Evaluation, Diagnosis, and Staging of Cutaneous Lymphoma”. Dermatologic Clinics. 33 (4): 643–654. doi:10.1016/j.det.2015.06.001. ISSN 0733-8635.
  29. ↑ Howlader N, Noone AM, Krapcho M, Garshell J, Miller D, Altekruse SF, Kosary CL, Yu M, Ruhl J, Tatalovich Z,Mariotto A, Lewis DR, Chen HS, Feuer EJ, Cronin KA (eds). SEER Cancer Statistics Review, 1975-2011, National Cancer Institute. Bethesda, MD, http://seer.cancer.gov/csr/1975_2011/, based on November 2013 SEER data submission, posted to the SEER web site, April 2014.

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