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| {{DISPLAYTITLE:B lymphoblastic leukaemia/lymphoma with KMT2A rearrangement}} | | {{DISPLAYTITLE:B lymphoblastic leukaemia/lymphoma with KMT2A rearrangement}} |
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| [[HAEM5:Table_of_Contents|Haematolymphoid Tumours (WHO Classification, 5th ed.)]] | | [[HAEM5:Table_of_Contents|Haematolymphoid Tumours (WHO Classification, 5th ed.)]] |
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| {{Under Construction}} | | {{Under Construction}} |
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| <blockquote class='blockedit'>{{Box-round|title=Content Update To WHO 5th Edition Classification Is In Process; Content Below is Based on WHO 4th Edition Classification|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:B-Lymphoblastic Leukemia/Lymphoma with t(v;11q23.3); KMT2A-Rearranged]]. | | <blockquote class="blockedit">{{Box-round|title=Content Update To WHO 5th Edition Classification Is In Process; Content Below is Based on WHO 4th Edition Classification|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:B-Lymphoblastic Leukemia/Lymphoma with t(v;11q23.3); KMT2A-Rearranged]]. |
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| |Subtype(s) | | |Subtype(s) |
| |B lymphoblastic leukaemia/lymphoma with KMT2A rearrangement | | |B lymphoblastic leukaemia/lymphoma with KMT2A rearrangement |
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| ==Definition / Description of Disease==
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| B lymphoblastic leukemia/ lymphoma is the most common childhood cancer. Despite cure rates exceeding 90%, it remains an important cause of morbidity and mortality in adults and young children, especially when the disease relapses. It is a neoplasm of precursor cells (lymphoblasts) that are committed to the B-cell lineage. Blast cells are small to medium sized, with scant cytoplasm and inconspicuous nucleoli, mostly involving the bone marrow and peripheral blood. Occasionally, however, it can present with involvement of nodal and extra nodal sites (eg. lymph nodes and skin), at which point, it is more accurately referred to as B lymphoblastic lymphoma.
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| This class of the disease harbors a translocation between the MLL/KMT2A at 11q23 and any one of the large number of fusion partners. Patients with deletions of the MLL/KMT2A locus are not included in this group.
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| ==Synonyms / Terminology==
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| MLL = KMT2A
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| With extensive bone marrow and peripheral blood involvement, B lymphoblastic leukemia is the most appropriate term. If, however, the disease presents as a mass lesion with minimal involvement of the bone marrow and peripheral blood, the term lymphoma should be used. When both sites are involved, the distinction between leukemia and lymphoma is arbitrary. A figure of 25% blasts in the bone marrow is used in some protocols as a threshold for defining leukemia.
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| ==Epidemiology / Prevalence==
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| Etiology While the etiology of MLL translocations is unknown, there is strong evidence suggesting that it may occur in utero. These leukemias frequently affect very young infants, and this translocation is sometimes detected in blood spots of patients who later develop the disease. The MLL rearrangement is also seen in 85% of secondary leukemias that occur in patients treated with topoisomerase II inhibitors.
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| Epidemiology / Prevalence B-LBL/L is primarily a disease of children. 75% of cases occur in children under six years of age. The worldwide incidence is estimated at 1-5/100,000 persons per year.
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| MLL-rearranged B-ALL is often detected in infant leukemia and accounts for ~2% of all childhood ALLs. The outcome of MLL-R infant ALL remains poor with an event-free survival of 28-36% (Andersson AK et al., 2015 to be quoted in excel sheet). “MLL (mixed-lineage-leukemia) gene rearrangements at 11q23 are present in 80% of all infant B-ALL cases and 10% of all childhood B-ALL [38,39].”
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| ==Clinical Features==
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| Put your text here and fill in the table <span style="color:#0070C0">(''Instruction: Can include references in the table. Do not delete table.'') </span>
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| {| class="wikitable"
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| |'''Signs and Symptoms'''
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| |<span class="blue-text">EXAMPLE:</span> Asymptomatic (incidental finding on complete blood counts)
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| <span class="blue-text">EXAMPLE:</span> B-symptoms (weight loss, fever, night sweats)
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| <span class="blue-text">EXAMPLE:</span> Fatigue
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| <span class="blue-text">EXAMPLE:</span> Lymphadenopathy (uncommon)
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| |'''Laboratory Findings'''
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| |<span class="blue-text">EXAMPLE:</span> Cytopenias
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| <span class="blue-text">EXAMPLE:</span> Lymphocytosis (low level)
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| |}
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| <blockquote class='blockedit'>{{Box-round|title=v4:Clinical Features|The content below was from the old template. Please incorporate above.}}</blockquote>
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| A substantial proportion of congenital leukemias, a subset of infant leukemias, harbors a rearrangement of the MLL gene (Moschiano E et al., 2016) MLL rearranged leukemia is associated with certain phenotypic features that distinguish them from other types of leukemia. MLL leukemias tend to be more aggressive, especially in infants, and more frequently present with hyperleukocytosis and central nervous system involvement.
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| <blockquote class="blockedit">
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| <center><span style="color:Maroon">'''End of V4 Section'''</span>
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| ----
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| </blockquote>
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| ==Sites of Involvement==
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| Put your text here <span style="color:#0070C0">(''Instruction: Indicate physical sites; <span class="blue-text">EXAMPLE:</span> nodal, extranodal, bone marrow'') </span>
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| ==Morphologic Features==
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| ==Immunophenotype==
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| Put your text here and fill in the table <span style="color:#0070C0">(''Instruction: Can include references in the table. Do not delete table.'') </span>
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| {| class="wikitable sortable"
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| !Finding!!Marker
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| |Positive (universal)||<span class="blue-text">EXAMPLE:</span> CD1
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| |Positive (subset)||<span class="blue-text">EXAMPLE:</span> CD2
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| |Negative (universal)||<span class="blue-text">EXAMPLE:</span> CD3
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| |Negative (subset)||<span class="blue-text">EXAMPLE:</span> CD4
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| |} | | |} |
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| (use the "Cite" icon at the top of the page) <span style="color:#0070C0">(''Instructions: Add each reference into the text above by clicking where you want to insert the reference, selecting the “Cite” icon at the top of the wiki page, and using the “Automatic” tab option to search by PMID to select the reference to insert. If a PMID is not available, such as for a book, please use the “Cite” icon, select “Manual” and then “Basic Form”, and include the entire reference. To insert the same reference again later in the page, select the “Cite” icon and “Re-use” to find the reference; DO NOT insert the same reference twice using the “Automatic” tab as it will be treated as two separate references. The reference list in this section will be automatically generated and sorted''</span><span style="color:#0070C0">''.''</span><span style="color:#0070C0">)</span> <references /> | | (use the "Cite" icon at the top of the page) <span style="color:#0070C0">(''Instructions: Add each reference into the text above by clicking where you want to insert the reference, selecting the “Cite” icon at the top of the wiki page, and using the “Automatic” tab option to search by PMID to select the reference to insert. If a PMID is not available, such as for a book, please use the “Cite” icon, select “Manual” and then “Basic Form”, and include the entire reference. To insert the same reference again later in the page, select the “Cite” icon and “Re-use” to find the reference; DO NOT insert the same reference twice using the “Automatic” tab as it will be treated as two separate references. The reference list in this section will be automatically generated and sorted''</span><span style="color:#0070C0">''.''</span><span style="color:#0070C0">)</span> <references /> |
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| ==Notes== | | ==Notes== |
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| <nowiki>*</nowiki>''Citation of this Page'': “B lymphoblastic leukaemia/lymphoma with KMT2A rearrangement”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:B_lymphoblastic_leukaemia/lymphoma_with_KMT2A_rearrangement</nowiki>. | | <nowiki>*</nowiki>''Citation of this Page'': “B lymphoblastic leukaemia/lymphoma with KMT2A rearrangement”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:B_lymphoblastic_leukaemia/lymphoma_with_KMT2A_rearrangement</nowiki>. |
| [[Category:HAEM5]][[Category:DISEASE]][[Category:Diseases B]] | | [[Category:HAEM5]] |
| | [[Category:DISEASE]] |
| | [[Category:Diseases B]] |
