Lymphoid leukemia#T-cell leukemias

Historically, they have been most commonly divided by the stage of maturation at which the clonal (neoplastic) lymphoid population stopped maturing:{{cn|date=November 2021}}

• Acute lymphoblastic leukemia
• Chronic lymphocytic leukemia

However, the influential WHO Classification (published in 2001) emphasized a greater emphasis on cell lineage. To this end, lymphoid leukemias can also be divided by the type of cells affected:

• B-cell leukemia
• T-cell leukemia
• NK-cell leukemia

The most common type of lymphoid leukemia is B-cell chronic lymphocytic leukemia.

B-cell leukemia describes several different types of lymphoid leukemia which affect B cells.

Other types include (with ICD-O code):

• 9826/3 – Acute lymphoblastic leukemia, mature B-cell type
• 9833/3 – B-cell prolymphocytic leukemia
• 9940/3 – Hairy cell leukemia

T-cell leukemia describes several different types of lymphoid leukemias which affect T cells.{{cn|date=November 2021}}

The most common T-cell leukemia is precursor T-cell lymphoblastic leukemia. It causes 15% of acute leukemias in childhood, and also 40% of lymphomas in childhood. It is most common in adolescent males. Its morphology is identical to that of precursor B-cell lymphoblastic leukemia. Cell markers include TdT, CD2, CD7. It often presents as a mediastinal mass because of involvement of the thymus. It is highly associated with NOTCH1 mutations.

Other types include:

• Large granular lymphocytic leukemia
• Adult T-cell leukemia/lymphoma
• T-cell prolymphocytic leukemia

In practice, it can be hard to distinguish T-cell leukemia from T-cell lymphoma, and they are often grouped together.

Aggressive NK-cell leukemia (ANKL) is a lymphoid leukemia that is a deficiency NK cells. Not very much is known about this disease due to its rarity, but it is highly aggressive. A majority of patients with NK cell leukemia die within a year of diagnosis, and for ANKL in particular, half of patients die within two months.{{cite journal | vauthors = Suzuki R, Suzumiya J, Yamaguchi M, Nakamura S, Kameoka J, Kojima H, Abe M, Kinoshita T, Yoshino T, Iwatsuki K, Kagami Y, Tsuzuki T, Kurokawa M, Ito K, Kawa K, Oshimi K | title = Prognostic factors for mature natural killer (NK) cell neoplasms: aggressive NK cell leukemia and extranodal NK cell lymphoma, nasal type | journal = Ann. Oncol. | volume = 21 | issue = 5 | pages = 1032–40 | date = May 2010 | pmid = 19850638 | doi = 10.1093/annonc/mdp418 | doi-access = free }}

The requirements for diagnosing ANKL are as follows:{{cite journal | vauthors = Oshimi K | title = Leukemia and lymphoma of natural killer lineage cells | journal = Int. J. Hematol. | volume = 78 | issue = 1 | pages = 18–23 | date = July 2003 | pmid = 12894846 | doi = 10.1007/bf02983235| s2cid = 24785150 }}

1. Immature-looking NK cells
2. Certain immunophenotypes{{cite journal | vauthors = Landay AL, Muirhead KA | title = Procedural guidelines for performing immunophenotyping by flow cytometry | journal = Clin. Immunol. Immunopathol. | volume = 52 | issue = 1 | pages = 48–60 | date = July 1989 | pmid = 2656019 | doi = 10.1016/0090-1229(89)90192-x}}
3. Germline configuration genes: TCR-β and IgH
4. Restricted cytotoxicity

The T-cell receptor (TCR) is an important factor when ANKL is being diagnosed along with T-cell leukemia. The TCR gene transcripts are normally positive for ANKL. {{cite journal | vauthors = Hong M, Lee T, Young Kang S, Kim SJ, Kim W, Ko YH | title = Nasal-type NK/T-cell lymphomas are more frequently T rather than NK lineage based on T-cell receptor gene, RNA, and protein studies: lineage does not predict clinical behavior | journal = Mod. Pathol. | volume = 29 | issue = 5 | pages = 430–43 | date = May 2016 | pmid = 27015135 | doi = 10.1038/modpathol.2016.47 | doi-access = free }} Current Research is attempting to find the causation of ANKL. So far, the researchers have concluded that lineage of the T-cell receptor gene does not predict the behavior of the disease.

ANKL is treated similarly to most B-cell lymphomas. Anthracycline-containing chemotherapy regimens are commonly offered as the initial therapy. Some patients may receive a stem cell transplant. {{cite journal | vauthors = Mercadal S, Briones J, Xicoy B, Pedro C, Escoda L, Estany C, Camós M, Colomo L, Espinosa I, Martínez S, Ribera JM, Martino R, Gutiérrez-García G, Montserrat E, López-Guillermo A | title = Intensive chemotherapy (high-dose CHOP/ESHAP regimen) followed by autologous stem-cell transplantation in previously untreated patients with peripheral T-cell lymphoma | journal = Ann. Oncol. | volume = 19 | issue = 5 | pages = 958–63 | date = May 2008 | pmid = 18303032 | doi = 10.1093/annonc/mdn022 | doi-access = free }}{{cite journal | vauthors = Reimer P, Schertlin T, Rüdiger T, Geissinger E, Roth S, Kunzmann V, Weissinger F, Nerl C, Schmitz N, Müller-Hermelink HK, Wilhelm M | title = Myeloablative radiochemotherapy followed by autologous peripheral blood stem cell transplantation as first-line therapy in peripheral T-cell lymphomas: first results of a prospective multicenter study | journal = Hematol. J. | volume = 5 | issue = 4 | pages = 304–11 | date = 2004 | pmid = 15297846 | doi = 10.1038/sj.thj.6200359 }}

Overall survival depends on the stage of the cancer when treatment is initiated, and on a composite of numerous risk factors. The median time from diagnosis to death is less than 1 year in patients overall. Patients diagnosed early and/or with fewer risk factors can sometimes enter complete remission and expect much longer survival.

Flow cytometry is a diagnostic tool in order to count/visualize the amount of lymphatic cells in the body. T cells, B cells and NK cells are nearly impossible to distinguish under a microscope, therefore one must use a flow cytometer to distinguish them.

Several molecular tumor profiling protocols have been initiated in Europe (e.g., MOSCATO-01, iTHER, and ESMART) to identify actionable lesions for targeted treatment in specific subgroups of patients.{{cite journal | vauthors = Cordo V, Meijerink J | title = T-cell Acute Lymphoblastic Leukemia: A Roadmap to Targeted Therapies | journal = Blood Cancer Discovery | volume = 2 | pages = 19–31 |date=January 2021 | issue = 1 |doi = 10.1158/2643-3230.BCD-20-0093| pmid = 34661151 | pmc=8447273 | doi-access = free }}

Natural killer (NK) cell therapy is used in pediatrics for children with relapsed lymphoid leukemia. These patients normally have a resistance to chemotherapy, therefore, in order to continue on, must receive some kind of therapy. In some cases, NK cell therapy is a choice.{{cite journal | vauthors = Rubnitz JE, Inaba H, Kang G, Gan K, Hartford C, Triplett BM, Dallas M, Shook D, Gruber T, Pui CH, Leung W | title = Natural killer cell therapy in children with relapsed leukemia | journal = Pediatr Blood Cancer | volume = 62 | issue = 8 | pages = 1468–72 | date = August 2015 | pmid = 25925135 | pmc = 4634362 | doi = 10.1002/pbc.25555 }}

NK cells are known for their ability to eradicate tumor cells without any prior sensitization to them.{{Cite journal|pmid=26303618|pmc=4548900|year=2015|last1=Sakamoto|first1=N|title=Phase I clinical trial of autologous NK cell therapy using novel expansion method in patients with advanced digestive cancer|journal=Journal of Translational Medicine|volume=13|pages=277|last2=Ishikawa|first2=T|last3=Kokura|first3=S|last4=Okayama|first4=T|last5=Oka|first5=K|last6=Ideno|first6=M|last7=Sakai|first7=F|last8=Kato|first8=A|last9=Tanabe|first9=M|last10=Enoki|first10=T|last11=Mineno|first11=J|last12=Naito|first12=Y|last13=Itoh|first13=Y|last14=Yoshikawa|first14=T|doi=10.1186/s12967-015-0632-8 |doi-access=free }} One problem when using NK cells in order to fight off lymphoid leukemia is the fact that it is hard to amount enough of them to be effective. One can receive donations of NK cells from parents or relatives through bone marrow transplants. There are also the issues of cost, purity and safety.{{Cite journal| doi=10.1615/CritRevOncog.2014011091| pmid=24941379| pmc=4066212| title=NK Cells in Therapy of Cancer| journal=Critical Reviews in Oncogenesis| volume=19| issue=1–2| pages=133–41| year=2014| last1=Bachanova| first1=Veronika| last2=Miller| first2=Jeffrey S.}} Unfortunately, there is always the possibility of Graft vs host disease while transplanting bone marrow.

NK cell therapy is a possible treatment for many different cancers such as Malignant glioma.{{Cite journal| doi=10.1016/j.molmed.2011.03.004| pmid=21507717| title=Immunotherapy in gliomas: Limitations and potential of natural killer (NK) cell therapy| journal=Trends in Molecular Medicine| volume=17| issue=8| pages=433–41| year=2011| last1=Ogbomo| first1=Henry| last2=Cinatl| first2=Jindrich| last3=Mody| first3=Christopher H.| last4=Forsyth| first4=Peter A.}}

{{reflist}}

{{Medical resources

| DiseasesDB =

| ICD10 = {{ICD10|C|91||c|81}}

| ICD9 = {{ICD9|204}}

| ICDO =

| OMIM =

| MedlinePlus =

| eMedicineSubj =

| eMedicineTopic =

| MeshID = D007945

| SNOMED CT = 32280000

}}

{{Lymphoid malignancy}}

Category:Leukemia