Diagnosis of Pediatric-Type Follicular Lymphoma in Young Adults (Own Data)
Aim. Pathomorphological, immunophenotypical and clinical characteristics of a new clinico-morphological form of pediatric-type follicular lymphoma (FL) in young adults discovered in 2008 (WHO classification). Background. FL is a heterogeneous disease according to its morphological, immunophenotyp...
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Practical Medicine Publishing House
2017-01-01
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| Series: | Клиническая онкогематология |
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| Online Access: | http://bloodjournal.ru/wp-content/uploads/2017/01/7_reduced.pdf |
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| author | AM Kovrigina LV Plastinina SK Kravchenko ES Nesterova TN Obukhova |
| author_facet | AM Kovrigina LV Plastinina SK Kravchenko ES Nesterova TN Obukhova |
| author_sort | AM Kovrigina |
| collection | DOAJ |
| description | Aim. Pathomorphological, immunophenotypical and clinical characteristics of a new clinico-morphological form of pediatric-type follicular lymphoma (FL) in young adults discovered in 2008 (WHO classification).
Background. FL is a heterogeneous disease according to its morphological, immunophenotypical and molecular-genetic characteristics. FL de novo includes transformed FL, FL without t(14;18), FL with diffuse growth associated with del(1p.36) and TNFRSF14 mutation. Pediatric-type FL in young adults is poorly studied; and it is especially interesting because of its clinical diversity and molecular-genetic heterogeneity of FL, in general.
Methods. Biopsy materials taken from 5 patients (aged 18–25 years; median age: 22 years; the female/male ratio 3:2) were included in the study; all patients were examined, diagnosed and treated in the Hematology Research Center over the period from 2012 to 2016. Clinical stage I with isolated involvement a palatine tonsil or an inguinal lymph node was diagnosed in 4/5 patients; clinical stage II with involvement of a palatine tonsil and cervical lymph node was diagnosed in 1/5 patients. Morphological, immunophenotypical and FISH tests were performed with paraffin blocks.
Results. The morphological pattern was typical for FL 3B (n = 2) and FL 3 with blastoid nucleus morphology (n = 3). Immunophenotypical features demonstrated an intermediate position between FL 3 de novo and transformed FL 3. No BCL-2 rearrangement was detected in any observation.
Conclusion. The comparison of our data on characteristics of pediatric-type FL with those published in the literature demonstrated that lack or weak expression (< 30 % of tumor substrate cells) of MUM1 was the key feature of the experimental group of young adults with pediatric-type FL. This, in turn, indicates the absence of IRF4 rearrangements and possible presence of other genetic abnormalities. The clinical, morphological, and immunophenotypical characteristics broaden the FL heterogeneity spectrum in young adults. |
| format | Article |
| id | doaj-art-0fb5772c032b4ddab4259c677fcaaad4 |
| institution | OA Journals |
| issn | 1997-6933 2500-2139 |
| language | Russian |
| publishDate | 2017-01-01 |
| publisher | Practical Medicine Publishing House |
| record_format | Article |
| series | Клиническая онкогематология |
| spelling | doaj-art-0fb5772c032b4ddab4259c677fcaaad42025-08-20T01:58:48ZrusPractical Medicine Publishing HouseКлиническая онкогематология1997-69332500-21392017-01-01101526010.21320/2500-2139-2017-10-1-52-60Diagnosis of Pediatric-Type Follicular Lymphoma in Young Adults (Own Data)AM Kovrigina0LV Plastinina1SK Kravchenko2ES Nesterova3TN Obukhova4Hematology Research Center under the Ministry of Health of the Russian Federation, 4а Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167Hematology Research Center under the Ministry of Health of the Russian Federation, 4а Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167Hematology Research Center under the Ministry of Health of the Russian Federation, 4а Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167Hematology Research Center under the Ministry of Health of the Russian Federation, 4а Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167Hematology Research Center under the Ministry of Health of the Russian Federation, 4а Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167Aim. Pathomorphological, immunophenotypical and clinical characteristics of a new clinico-morphological form of pediatric-type follicular lymphoma (FL) in young adults discovered in 2008 (WHO classification). Background. FL is a heterogeneous disease according to its morphological, immunophenotypical and molecular-genetic characteristics. FL de novo includes transformed FL, FL without t(14;18), FL with diffuse growth associated with del(1p.36) and TNFRSF14 mutation. Pediatric-type FL in young adults is poorly studied; and it is especially interesting because of its clinical diversity and molecular-genetic heterogeneity of FL, in general. Methods. Biopsy materials taken from 5 patients (aged 18–25 years; median age: 22 years; the female/male ratio 3:2) were included in the study; all patients were examined, diagnosed and treated in the Hematology Research Center over the period from 2012 to 2016. Clinical stage I with isolated involvement a palatine tonsil or an inguinal lymph node was diagnosed in 4/5 patients; clinical stage II with involvement of a palatine tonsil and cervical lymph node was diagnosed in 1/5 patients. Morphological, immunophenotypical and FISH tests were performed with paraffin blocks. Results. The morphological pattern was typical for FL 3B (n = 2) and FL 3 with blastoid nucleus morphology (n = 3). Immunophenotypical features demonstrated an intermediate position between FL 3 de novo and transformed FL 3. No BCL-2 rearrangement was detected in any observation. Conclusion. The comparison of our data on characteristics of pediatric-type FL with those published in the literature demonstrated that lack or weak expression (< 30 % of tumor substrate cells) of MUM1 was the key feature of the experimental group of young adults with pediatric-type FL. This, in turn, indicates the absence of IRF4 rearrangements and possible presence of other genetic abnormalities. The clinical, morphological, and immunophenotypical characteristics broaden the FL heterogeneity spectrum in young adults.http://bloodjournal.ru/wp-content/uploads/2017/01/7_reduced.pdfpediatric-type follicular lymphomafollicular lymphomayoung adultspathomorphologyimmunohictochemistryMUM1 |
| spellingShingle | AM Kovrigina LV Plastinina SK Kravchenko ES Nesterova TN Obukhova Diagnosis of Pediatric-Type Follicular Lymphoma in Young Adults (Own Data) Клиническая онкогематология pediatric-type follicular lymphoma follicular lymphoma young adults pathomorphology immunohictochemistry MUM1 |
| title | Diagnosis of Pediatric-Type Follicular Lymphoma in Young Adults (Own Data) |
| title_full | Diagnosis of Pediatric-Type Follicular Lymphoma in Young Adults (Own Data) |
| title_fullStr | Diagnosis of Pediatric-Type Follicular Lymphoma in Young Adults (Own Data) |
| title_full_unstemmed | Diagnosis of Pediatric-Type Follicular Lymphoma in Young Adults (Own Data) |
| title_short | Diagnosis of Pediatric-Type Follicular Lymphoma in Young Adults (Own Data) |
| title_sort | diagnosis of pediatric type follicular lymphoma in young adults own data |
| topic | pediatric-type follicular lymphoma follicular lymphoma young adults pathomorphology immunohictochemistry MUM1 |
| url | http://bloodjournal.ru/wp-content/uploads/2017/01/7_reduced.pdf |
| work_keys_str_mv | AT amkovrigina diagnosisofpediatrictypefollicularlymphomainyoungadultsowndata AT lvplastinina diagnosisofpediatrictypefollicularlymphomainyoungadultsowndata AT skkravchenko diagnosisofpediatrictypefollicularlymphomainyoungadultsowndata AT esnesterova diagnosisofpediatrictypefollicularlymphomainyoungadultsowndata AT tnobukhova diagnosisofpediatrictypefollicularlymphomainyoungadultsowndata |