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HAEM5:T-prolymphocytic leukaemia (view source)

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[[HAEM5:Table_of_Contents|Haematolymphoid Tumours (5th ed.)]]

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(''General Instructions – The main focus of these pages is the clinically significant genetic alterations in each disease type. Use [https://www.genenames.org/ HUGO-approved gene names and symbols] (italicized when appropriate), [https://varnomen.hgvs.org/ HGVS-based nomenclature for variants], as well as generic names of drugs and testing platforms or assays if applicable. Please complete tables whenever possible and do not delete them (add N/A if not applicable in the table and delete the examples); to add (or move) a row or column to a table, click nearby within the table and select the > symbol that appears to be given options. Please do not delete or alter the section headings. The use of bullet points alongside short blocks of text rather than only large paragraphs is encouraged. Additional instructions below in italicized blue text should not be included in the final page content. Please also see'' ''[[Author_Instructions]]'' ''and [[Frequently Asked Questions (FAQs)|FAQs]] as well as contact your [[Leadership|Associate Editor]] or [mailto:CCGA@cancergenomics.org Technical Support])''

==Primary Author(s)*==

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~~Put your text here~~ ~~(''EXAMPLE:'' Jane Smith, PhD)~~

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Parastou Tizro, MD

==WHO Classification of Disease==

(Will be autogenerated; Book will include name of specific book and have a link to the online WHO site)

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==Definition / Description of Disease==

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~~Put your text here ~~(~~''Instructions: Brief description~~ of ~~approximately one paragraph~~ - ~~include disease context relative~~ to ~~other WHO classification categories~~, ~~diagnostic criteria if applicable, and differential diagnosis if applicable~~. ~~Other classifications can be referenced for comparison.'') ~~

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T-prolymphocytic leukemia (T-PLL) is a clonal proliferation of small to medium-sized prolymphocytes with a mature post thymic T-cell phenotype characterized by the juxtaposition of TCL1A or MTCP1 to a TR locus, most often the TRA/TRD locus.

==Synonyms / Terminology==

Put your text here (''Instructions: Include currently used terms and major historical ones, adding “(historical)” after the latter.'')

==Epidemiology / Prevalence==

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~~Put your text here~~

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T-PLL is rare, accounting for approximately 2% of cases of mature lymphoid leukemias in adults aged over 30 years. It predominantly affects the elderly (median age: 65 years, range: 30–94 years) with a slight male predominance (M:F ratio: 1.33:1).

==Clinical Features==

Put your text here and fill in the table (''Instruction: Can include references in the table. Do not delete table.'')

{| class="wikitable"

|'''Signs and Symptoms'''

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|~~EXAMPLE: Asymptomatic~~ (~~incidental finding on complete blood counts~~)

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|Hepatosplenomegaly (Frequently observed)

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~~EXAMPLE: B-symptoms~~ (~~weight loss, fever, night sweats~~)

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Generalized lymphadenopathy with slightly enlarged lymph nodes (Frequently observed

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Cutaneous involvement (20%)

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Malignant effusions (15%)

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~~EXAMPLE: Lymphadenopathy~~ (~~uncommon~~)

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Asymptomatic and indolent phase (30% of cases)

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|'''Laboratory Findings'''

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|~~~~EXAMPLE:<~~/~~span> Cytopenias

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|Anaemia and thrombocytopenia

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Marked lymphocytosis > 100 × 10^9/L (75% of cases)

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EXAMPLE: Lymphocytosis (low level)

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==Sites of Involvement==

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~~Put your text here (''Instruction: Indicate physical sites; EXAMPLE: nodal, extranodal~~, bone marrow~~'') ~~

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Peripheral blood, bone marrow, spleen, liver, lymph node, and sometimes skin and serosa

==Morphologic Features==

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~~Put your text here (''Instructions: Brief description~~ of ~~typically approximately one paragraph'') ~~

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Blood smears display anemia, thrombocytopenia, and leukocytosis, predominantly of atypical lymphocytes. Bone marrow aspirates show aggregates of neoplastic lymphoid cells.

==Immunophenotype==

Put your text here and fill in the table (''Instruction: Can include references in the table. Do not delete table.'')

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!Finding!!Marker

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|Positive (universal)||~~EXAMPLE: CD1~~

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|Positive (universal)||CD2, CD3 (may be weak), CD5, CD7

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|Positive (subset)||

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|Positive (subset)||CD4 (in some cases CD4+/CD8+ or CD4-/CD8+), CD52

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|Negative (universal)||

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|Negative (universal)||TdT, CD1a

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|Negative (subset)||

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|Negative (subset)||CD8 (in some cases CD4+/CD8+ or CD4-/CD8+)

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==Chromosomal Rearrangements (Gene Fusions)==

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!Notes

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|~~EXAMPLE:~~ t(9;22)(~~q34~~;~~q11.2~~)||~~EXAMPLE: 3'ABL1~~ / ~~5'BCR~~||EXAMPLE: der(22)||EXAMPLE: 20% (COSMIC)

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|t(14;14)(q11;q32)||TCL1A/TRD||EXAMPLE: der(22)||EXAMPLE: 20% (COSMIC)

EXAMPLE: 30% (add reference)

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|~~EXAMPLE:~~ Yes

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|Yes

|EXAMPLE: No

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|~~EXAMPLE:~~ Yes

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|Yes

|EXAMPLE:

The t(9;22) is diagnostic of CML in the appropriate morphology and clinical context (add reference). This fusion is responsive to targeted therapy such as Imatinib (Gleevec) (add reference).

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|-

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|t(X;14)(q28;q11.2)

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|MTCP1/TRD

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|Low

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|Yes

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==Individual Region Genomic Gain / Loss / LOH==

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!Therapeutic Significance (Yes, No or Unknown)

!Notes

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~~|EXAMPLE:~~

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7

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~~|EXAMPLE: Loss~~

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~~|EXAMPLE:~~

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~~chr7:1-159,335,973 [hg38]~~

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~~|EXAMPLE:~~

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~~chr7~~

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~~|EXAMPLE: Yes~~

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~~|EXAMPLE: Yes~~

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~~|EXAMPLE: No~~

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~~|EXAMPLE:~~

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Presence of monosomy 7 (or 7q deletion) is sufficient for a diagnosis of AML with MDS-related changes when there is ≥20% blasts and no prior therapy (add reference). Monosomy 7/7q deletion is associated with a poor prognosis in AML (add reference).

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|EXAMPLE:

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|EXAMPLE: Gain

|EXAMPLE:

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~~chr8:1-145,138,636 [hg38]~~

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|EXAMPLE:

chr8

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<nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page. If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage). Additional global feedback or concerns are also welcome.

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[[Category:HAEM5]][[Category:DISEASE]][[Category:Diseases T]]

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[[Category:HAEM5]]

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[[Category:DISEASE]]

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[[Category:Diseases T]]

Parastou.Tizro

15

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