The recent identification among non-Hodgkin's malignant lymphomas of a high-grade malignancy entity of possible thymic origin and defined as lymphoblastic convoluted-cell lymphoma allowed the morphologic and radiological diagnosis of nine cases of this disease. Males outnumbered females (6/9) and the age ranged from 6 to 54 years. The histologic diagnosis relied upon the characteristic non cohesive cellularity as well as upon the peculiar cribriform nuclear structure, the convoluted appearance of the nuclei, the anisokaryosis, the paucity of the cytoplasm and the minimal size of the nucleoli. The anatomical distribution of the disease was ascertained in the lymph nodes, spleen, liver and several other extranodal sites. Bone marrow invasion was observed in seven cases and presence of lymphoma cells in the peripheral blood in six. A thymic mediastinal mass was present radiologically in seven cases and lymphography was positive in five. Follow-up data revealed death of four patients within 10 months and survival of two up to 17 months. The cellular morphology of the malignant lymphoma seems to be sufficiently diagnostic. This entity deserves an autonomous position within the classifications of malignant lymphomas as it appears in the functional one proposed by Lukes and Collins (21) as well as in the Kiel classification (13).
Get full access to this article
View all access options for this article.
References
1.
BonadonnaG.: La classificazione in stadi della malattia di Hodgkin. In: I Linfomi Maligni, pp. 153–158, BucalossiP., VeronesiU., BonadonnaG., EmanuelliH. (eds.), C.E.A., Milano, 1974.
2.
BorellaL., SenL.: T cell surface markers on lymphoblasts from acute lymphocytic leukemia.J. Immun., 111: 1257–1260, 1973.
3.
BorellaL., SenL.: T- and B- lymphocytes and lymphoblasts in untreated acute lymphocytic leukemia.Cancer, 34: 646–654, 1974.
4.
BraylanR. C., RappaportH.: Tissue immunoglobulins in nodular lymphomas as compared with reactive follicular hyperplasias.Blood, 42: 579–589, 1973.
5.
BroomeJ. D., Zucker-FranklinD., WeinerM. S., BiancoC., NussenzweigV.: Leukemic cells with membrane properties of thymus-derived (T) lymphocytes in a case of Sézar'ys syndrome: morphologic and immunologic studies.Clin. Immunol. Immunopath., 1: 319–329, 1973.
6.
BrouetJ. C., FlandrinG., SeligmanM.: Indications of the thymus- derived nature of the proliferating cells in six patients with Sézary's syndrome.New Eng. J. Med., 289: 341–344, 1973.
7.
BrownG., GraevesM. F., ListerT. A., RapsonN., PapamichaelM.: Expression of human T and B lymphocyte cell-surface markers on leukaemic cells.Lancet, II: 753–755, 1974.
8.
BrownleeT. R., MuradT. M.: Ultrastructure of mycosis fungoides.Cancer, 26: 686–698, 1970.
9.
ButlerJ. J.: Histopathology of malignant lymphomas and Hodgkin's disease.In: Leukemia Lymphoma, pp. 123–142, Year Book Medical Publishers, Chicago, 1970.
10.
CatovskyD., GalettoJ., OkosA., GaltonD. A. G., WiltshawE., StathopoulosG.: Prolymphocytic leukaemia of B and T cell type.Lancet, II: 232–234, 1973.
DorfmanR. F.: Letter to the Editor.Lancet, I: 1295–1296, 1974.
13.
Gérard-MarchantR., HamlinI., LennertK., RilkeF., StansfeldA. G., van UnnikJ.A.M.: Letter to the Editor.Lancet, II: 406–408, 1974.
14.
HansenJ. A., GoodR. A.: Malignant disease of the lymphoid system in immunological perspective.Human Pathol., 5: 567–599, 1974.
15.
KaplanJ., MastrangeloR., PetersonW. D.Jr.: Childhood lymphoblastic lymphoma, a cancer of thymus-derived lymphocytes.Cancer Res., 34: 521–525, 1974.
16.
KerseyJ. H., SabadA., Gajl-PeczalskaK., HallgrenH. M., YunisE. J., NesbitM. E.: Acute lymphoblastic leukemic cells with T (thymus-derived) lymphocyte markers.Science, 182: 1355–1356, 1973.
17.
LattuadaA., MilaniF.: Quadro clinico ed evoluzione dei linfomi maligni linfo-istiocitari. In: I Linfomi Maligni, pp. 95–109, BucalossiP., VeronesiU., BonadonnaG., EmanuelliH. (eds.), C.E.A., Milano1974.
18.
LennertK.: Lymphknoten. Bandteil A. Cytologie und Lymphadenitis. In: Handbuch der speziellen pathologischen Anatomie und Histologie.UehlingerE. (ed.), Springer Verlag, Berlin - New York, 1961.
19.
LennertK., MohriN.: Zur Pathologie der Leukämien und malignen Lymphome im Kindesalter.Verh. Deutsch. Ges. Path., 55: 216–269, 1971.
20.
LilleI., DesplacesA., MeeusL., SaracenoR. T.: Thymus-derived proliferating lymphocytes in chronic lymphocytic leukemia.Lancet, II: 263–264, 1973.
21.
LukesR. J., CollinsR. D.: Immunologic characterization of human malignant lymphomas.Cancer, 34: 1488–1503, 1974.
22.
LutznerM. A., HobbsJ. W., HorvathP.: Ultrastructure of abnormal cells in Sézary's syndrome, mycosis fungoides and parapsoriasis en plaque.Arch. Derm., 103: 375–386, 1971.
23.
LutznerM. A., EmeritI., DurepaireR., FlandrinG., GrupperG., PrunierasM.: Cytogenetic, cytophotometric and ultrastructural study of large cerebriform cells of the Sézary's syndrome and description of a small-cell variant.J. Nat. Cancer Inst., 50: 1145–1162, 1973.
24.
MoranE. M.: Staging classification for non-Hodgkin's lymphomas. In: I Linfomi Maligni. pp. 159–166, BucalossiP., VeronesiU., BonadonnaG., EmanuelliH. (eds.), C.E.A., Milano, 1974.
25.
MusumeciR., FarinaF. B., RabbiA.: L'esame linfoadenografico in linfomi maligni. In: I Linfomi Maligni, pp. 119–128, BucalossiP., VeronesiU., BonadonnaG., EmanuelliH. (eds.), C.E.A., Milano, 1974.
26.
NosanchukI. S., SchnitzerB.: Follicular hyperplasia in lymph nodes from patient with rheumatoid arthritis.A. clinico-pathologic study. Cancer, 24: 343–354, 1969.
27.
PiessensW. F., SchurP. H., MoloneyW. C., ChurchillW. H.: Lymphocyte surface immunoglobulins. Distribution and frequency in lymphoproliferative diseases.New Eng. I. Med., 288: 176–180, 1973.
28.
PilottiS., RilkeF.: La diagnosi citologica nella patologia linfonodale.Tumori, 58: 289–317, 1972.
29.
RappaportH.: Tumors of the hematopoietic system.A.F.I.P., Washinghton D.C., 1966.
30.
RappaportH.: Nouveaux concepts dans la classification des hémopathies malignes.Bull. Cancer, 61: 11–62, 1974.
31.
RappaportH., ThomasL. B.: Mycosis fungoides: the pathology of extracutaneous involvement.Cancer, 34: 1198–1229, 1974.
32.
RilkeF.: Istopatologia dei linfomi maligni linfo-istiocitari: In I Linfomi Maligni, pp. 23–27, BucalossiP., VeronesiU., BonadonnaG., EmanuelliH. (eds.), C.E.A., Milano, 1974.
SmithJ. L., CleinG. P., BarkerC. P., CollinsR. D.: Characterization of malignant mediastinal lymphoid neoplasm (Sternberg sarcoma) as thymic in origin.Lancet, I: 74–77, 1973.
36.
SternbergG.: Leukosarcomatose und Myeloblasten-Leukämie.Beitr. Path.,61: 75, 1916.
37.
WilsonJ. D., NossalG. I. V.: Identification of human T and B lymphocytes in normal peripheral blood and in chronic lymphocytic leukaemia.Lancet, II: 788–791, 1971.
38.
ZucaliR., UslenghiC.: Quadro clinico ed evoluzione della malattia di Hodgkin. In: I Linfomi Maligni, pp. 77–93, BucalossiP., VeronesiU., BonadonnaG., EmanuelliH. (eds.), C.E.A., Milano, 1974.
