Malignancies and Outcome in Patients with Ataxia-Telangiectasia: Results From the French National Registry for Primary Immune Deficiencies.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1101-1101
Author(s):  
Felipe Suarez ◽  
Julien Beauté ◽  
NIzar Mahlaoui ◽  
Romain Micol ◽  
Olivier Hermine ◽  
...  
Keyword(s):  
T Cell ◽  
B Cell ◽  
Ataxia Telangiectasia ◽  
Hematological Malignancies ◽  
Morbidity And Mortality ◽  
Response To Treatment ◽  
Cell Phenotype ◽  
High Grade ◽  
Major Contributor ◽  
Immune Deficiencies

Abstract Abstract 1101 Poster Board I-123 Background Ataxia-Telangiectasia (A-T) is rare inherited condition characterized by progressive neurological impairment, oculocutaneous telangiectasias, susceptibility to infections and predisposition to cancer. A-T is due to bi-allelic mutations in the ATM gene located on chromosome 11q23. ATM encodes a multifunction serine-threonine kinase involved detection and repair of DNA double-strand breaks, and regulation of cell-cycle check-points and apoptosis. A-T patients present an increased susceptibility to antimitotic chemotherapy and radiation.. Cancer is a major contributor to morbidity and mortality in A-T and ATM mutation analysis suggests a correlation between the presence of bi-allelic loss-of–function mutations and earlier onset of cancer, mainly hematological malignancies (R. Micol et al., manuscript submitted). Here we analyze the prevalence, histological subtype and outcome in A-T patients presenting with malignancies. Methods Two-hundred and fifty A-T patients born between 1954 and 2005 and included in the registry held by the French National Reference Center for Primary Immune Deficiencies (CEREDIH, manuscript submitted) were retrospectively analyzed. Clinical and histological data was extracted from the CEREDIH database. Results Fifty-five patients (22% of the entire A-T cohort) presented with malignancies. Mean age at diagnosis of malignancy was 14.4 years (SD 9.8). Forty-nine patients (89%) presented with hematological malignancies and 6 (10.9%) with adenocarcinoma (3 mammary gland, 2 gastric, 1 thyroid). Hematological malignancies occurred earlier than carcinomas (mean 11.8 vs. 33.7 years respectively, p<001). High-grade Non-hodgkin lymphoma (NHL) was the most frequent hematological malignancy (31 patients, 56.3%) followed by Hodgkin's lymphoma (HL, 9 cases, 16.3%), acute lymphoblastic leukemia (ALL, 7 cases, 12,7%) and T-cell prolymphocytic leukaemia (T-PLL, 2 cases, 3.6%). Among NHL, 20 were of B-cell phenotype, including 4 cases of Burkitt's lymphoma. Three NHL were of T-cell phenotype and in 8 cases the phenotype was not available. Information on tumor staging was available for 18 NHL. Seventeen NHL were disseminated (Ann Arbor stage III/IV). Extranodal involvement was present in 14 NHL, absent in one case and unknown in 16. Six NHL (all B-cell) were EBV(+), 2 EBV(-) and in 23 cases the information was missing. Thirteen out of 14 NHL patients for whom treatment information was available received chemotherapy. Information on dosage was available for 11 patients. Five of them received a reduced dose. Three patients with ALL received chemotherapy including 1 who also received radiotherapy as part of conditioning for allogeneic HSCT. Of the 3 patients with carcinoma for whom information was available, 1 received chemotherapy only, one radiotherapy only and one both. Toxicity was recorded for 8 NHL patients (no toxicity in 2, hematological in 2, other in 4). Treatment response was recorded in 9 NHL patients (CR in 3, PR in 3 and no response in 3). Cause of death was recorded in 10 NHL patients: 7 patients died from tumor progression, 1 from infection and 2 from other causes. Mean survival after diagnosis of malignancy was 1.3 years for all histological subtypes. Comparison according to type of response to treatment showed that patients undergoing a major response had a longer survival than patients with minor or no response (mean survival 4.14 years for the former vs. 0.28 years for the latter, p<0.05). Discussion Malignancy is a major contributor to morbidity and mortality in A-T, occurring in 22% of the present cohort. Hematological malignancies, mainly high grade B-cell NHL, are the most frequent cancers observed (56.3% of the observed malignancies and 12.4% of the patients in the cohort). EBV is associated with a noticeable proportion of high grade B-cell lymphomas, implying a possible contribution of the underlying immune deficiency in the pathogenesis of NHL during A-T. Tolerance to treatment including radiotherapy is variable. Despite poor overall survival, several patients underwent prolonged remissions and experienced improved survival. These results underscore the need to treat A-T patients with malignancies providing proper supportive care, including prevention and treatment of infections and mucositis. Further analysis of ATM mutations is required to determine if tolerance and response to treatment is correlated with the genotype. Disclosures No relevant conflicts of interest to declare.

scholarly journals Histological Characteristics of Ataxia Telangiectasia Associated Lymphoproliferative Diseases. Results of the French Registry of Primary Immune Deficiencies

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 1634-1634
Author(s):  
Danielle Canioni ◽  
Nizar Mahlaoui ◽  
Chantal Andriamanga ◽  
Catherine Dubois d'Enghien ◽  
Jean-Philippe Jais ◽  
...  
Keyword(s):  
T Cell ◽  
B Cell ◽  
Ataxia Telangiectasia ◽  
Who Classification ◽  
High Grade ◽  
Cell Type ◽  
Lymphoproliferative Diseases ◽  
Post Transplant ◽  
Immune Deficiencies ◽  
Pathology Reports

Abstract Introduction Ataxia-telangiectasia (A-T) is a rare autosomal recessive disorder associated with mutations in the ATMgene and characterized by cerebellar ataxia, telangiectasia, immune defect, and a high incidence of lymphoid and solid cancers. We conducted a retrospective study of the patients with AT enrolled in the registry of the French National Reference Center for Primary Immune Deficiencies, in order to describe the incidence, subtypes, and outcomes of cancer and mainly of lymphoproliferative diseases (LPD) occurring in AT. Most of the LPD of this study were centrally reviewed by 2 expert hematopathologists. Patients & Results Sixty nine patients with cancers were identified among the 279 patients with AT of our cohort. Eight patients developed carcinomas, 8 acute leukemias, 3 T-cell prolymphocytic leukemias and 50 developed lymphomas. Among the lymphomas, 12 were classical Hodgkin's lymphomas (cHL), and 38 high-grade non-Hodgkin lymphomas (NHL, 26 of B-cell type, 4 of T-cell type, and 8 not phenotyped). We obtained a centralized histopathology review in 31 cases classified by pathology reports as cHL (n=6) and high-grade NHL (n=25). Cases were reviewed according to the WHO classification by H&E staining and immunohistochemistry. The presence of EBV was analyzed using antibodies to LMP and in situ hybridization for EBER. Concordance between the diagnosis established on pathology reports and the centralized review was excellent. All 6 cases of cHL were confirmed by the pathology review. The 25 cases initially diagnosed as high-grade NHL based on pathology reports fell into several WHO classification categories. Six corresponded to Burkitt's lymphomas (BL) and 12 to diffuse large B-cell lymphomas (DLBCL). Among the latter, 10 could be further classified according to cell-of-origin by the Hans algorithm as germinal center (GC, 2) and non-GC (8). Seven cases displayed polymorphic histological features reminiscent of post-transplant lymphoproliferative disorders (LPD), 5 polymorphic B cell LPD (pLPD), 1 with features of infectious mononucleosis (IM), another with features of plasmacytic hyperplasia LPD. EBV could be evaluated in 28 cases. All 6 cHL, 4 out of 11 DLBCL, all 5 pLPD, and the case of IM-like LPD were EBV positive by LMP and/or EBER staining. The 5 cases of BL that were evaluated were all EBV negative. Median age at diagnosis was 7.8 years [range 3 – 24], 14.9 [5.8 – 17.1], 12.2 [5.4 – 29] and 11 [6.3 – 17.7] for pLPD, cHL, BL and DLBCL respectively (p=ns). Median survival after diagnosis of lymphoma was 35, 8.6, 6 and 8 months for pLPD, cHL, BL and DLBCL respectively (p=ns). ATM mutation analysis was available for 20 cases (8 hypomorphic mutations, 12 loss-of-function mutations). There were no differences in age at diagnosis of lymphoma or survival according to the ATM mutation class or the EBV status. Conclusion B-cell malignancies including cHL and B-cell NHL are the most frequent malignancies in A-T and can be further classified as DLBCL, BL and cases with polymorphic features resembling post-transplant LPD. EBV seems to be associated with all cHL, approximately 50% of DLBCL and polymorphic LPD, but not with BL. The majority of DLBCL are non-GC type by immunohistochemistry. On this series of 31 patients, ATM mutation type was not associated with differences in type of lymphoma or outcome. Disclosures No relevant conflicts of interest to declare.

Incidence, Presentation, and Prognosis of Malignancies in Ataxia-Telangiectasia: A Report From the French National Registry of Primary Immune Deficiencies

2015 ◽  
Vol 33 (2) ◽  
pp. 202-208 ◽  
Author(s):  
Felipe Suarez ◽  
Nizar Mahlaoui ◽  
Danielle Canioni ◽  
Chantal Andriamanga ◽  
Catherine Dubois d'Enghien ◽  
...  
Keyword(s):  
T Cell ◽  
B Cell ◽  
Hodgkin Lymphoma ◽  
Ataxia Telangiectasia ◽  
Lymphoblastic Leukemia ◽  
Response To Treatment ◽  
Acute Leukemias ◽  
Prolymphocytic Leukemia ◽  
Increased Risk ◽  
Risk Of Cancer

Purpose Biallelic mutations in ATM cause ataxia-telangiectasia (AT), a rare inherited disease with a high incidence of cancer. Precise estimates of the risk, presentation, and outcomes of cancer in patients with AT need to be addressed in large series. Patients and Methods In this large retrospective cohort, 69 patients with cancers (24.5%) were identified among 279 patients with AT. Centralized review was performed on 60% of the lymphomas. Incidence rates were compared with the French population, and risk factors were analyzed. Results Eight patients developed acute leukemias (including four T-cell acute lymphoblastic leukemias), 12 developed Hodgkin lymphoma (HL), 38 developed non-Hodgkin lymphoma (NHL), three developed T-cell prolymphocytic leukemia (T-PLL), and eight developed carcinoma at a median age of 8.3, 10.6, 9.7, 24.2, and 31.4 years, respectively (P < .001). The majority of NHLs were aggressive B-cell NHL. Epstein-Barr virus was associated with all of the HLs and 50% of the NHLs. Overall survival was shorter in patients with AT who developed cancer compared with those who did not develop cancer (15 v 24 years, respectively; P < .001). Survival was improved in patients who achieved a major response to treatment (3.46 v 0.87 years for major v minor responses, respectively; P = .011). Immunodeficiency was associated with increased risk of cancer. ATM mutation type was associated with a difference in survival in the entire cohort but not with cancer incidence or cancer survival. Conclusion B-cell NHL, HL, and acute lymphoblastic leukemia occur at a high rate and earlier age than carcinomas in AT. T-PLLs are rarer than initially reported. Prognosis is poor, but patients may benefit from treatment with an improved survival.

scholarly journals Dual T cell– and B cell–intrinsic deficiency in humans with biallelic RLTPR mutations

2016 ◽  
Vol 213 (11) ◽  
pp. 2413-2435 ◽  
Author(s):  
Yi Wang ◽  
Cindy S. Ma ◽  
Yun Ling ◽  
Aziz Bousfiha ◽  
Yildiz Camcioglu ◽  
...  
Keyword(s):  
T Cells ◽  
T Cell ◽  
B Cells ◽  
B Cell ◽  
Cd4 T Cells ◽  
Ex Vivo ◽  
Cell Phenotype ◽  
Loss Of Function ◽  
Inborn Errors

Combined immunodeficiency (CID) refers to inborn errors of human T cells that also affect B cells because of the T cell deficit or an additional B cell–intrinsic deficit. In this study, we report six patients from three unrelated families with biallelic loss-of-function mutations in RLTPR, the mouse orthologue of which is essential for CD28 signaling. The patients have cutaneous and pulmonary allergy, as well as a variety of bacterial and fungal infectious diseases, including invasive tuberculosis and mucocutaneous candidiasis. Proportions of circulating regulatory T cells and memory CD4+ T cells are reduced. Their CD4+ T cells do not respond to CD28 stimulation. Their CD4+ T cells exhibit a "Th2" cell bias ex vivo and when cultured in vitro, contrasting with the paucity of "Th1," "Th17," and T follicular helper cells. The patients also display few memory B cells and poor antibody responses. This B cell phenotype does not result solely from the T cell deficiency, as the patients’ B cells fail to activate NF-κB upon B cell receptor (BCR) stimulation. Human RLTPR deficiency is a CID affecting at least the CD28-responsive pathway in T cells and the BCR-responsive pathway in B cells.

scholarly journals Epidemiology, clinical and cytological features of lymphoma in Boxer dogs

2019 ◽  
Vol 67 (2) ◽  
pp. 224-240
Author(s):  
Urszula Jankowska ◽  
Dariusz Jagielski ◽  
Michał Czopowicz ◽  
Rafał Sapierzyński
Keyword(s):  
T Cell ◽  
B Cell ◽  
Cell Lymphoma ◽  
Clinical Stage ◽  
T Cell Lymphoma ◽  
Low Grade ◽  
High Grade ◽  
Kiel Classification ◽  
Cytological Features ◽  
Boxer Dogs

The aim of this study was to evaluate the epidemiology, clinical and laboratory characteristics of canine lymphomas as well as some aspects of treatment outcomes. The study was conducted on Boxer dogs with lymphoma diagnosed by cytology and immunocytochemistry (CD3 and CD79 alpha). During the study period, lymphoma was diagnosed in 63 Boxers; 86.8% were T-cell (based on the Kiel classification: small clear cell lymphoma, pleomorphic small cell lymphoma, pleomorphic mixed T-cell lymphoma, pleomorphic large T-cell lymphoma, lymphoblastic lymphoma/acute lymphoblastic leukaemia) and 13.2% were B-cell lymphomas (according to the Kiel classification: B-cell chronic lymphocytic leukaemia, centroblastic/centroblastic polymorphic lymphoma). Overall survival (OS) was significantly longer in dogs with low-grade than with high-grade lymphoma (median OS of 6.8 and 4.7 months, respectively; P = 0.024). OS was not influenced by WHO clinical stage, WHO clinical substage, presence of splenomegaly, early administration of glucocorticoids or the time from the first presentation to the beginning of chemotherapy. There are no significant differences in clinical and laboratory parameters between low-grade and high-grade lymphomas. Boxer dogs are predisposed to T-cell lymphoma, with a predominance of high-grade tumour, especially pleomorphic, mixed small and large T-cell subtype. It is possible that Boxer dogs may respond less favourably to chemotherapy than patients of other breeds.

Direct comparisons of peripheral T-cell lymphoma with diffuse B-cell lymphoma of comparable histological grades--should peripheral T-cell lymphoma be considered separately?

1989 ◽  
Vol 7 (6) ◽  
pp. 725-731 ◽  
Author(s):  
A L Cheng ◽  
Y C Chen ◽  
C H Wang ◽  
I J Su ◽  
H C Hsieh ◽  
...  
Keyword(s):  
T Cell ◽  
B Cell ◽  
Induction Chemotherapy ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
T Cell Lymphoma ◽  
High Grade ◽  
Peripheral T Cell Lymphoma ◽  
Laboratory Features ◽  
Hodgkin's Lymphomas

Peripheral T-cell lymphoma (PTCL) forms a morphologically heterogeneous group of non-Hodgkin's lymphomas (NHL) with distinct immunophenotypes of mature T cells. Progress has been slow in defining specific clinicopathological entities to this particular group of NHL. In order to elucidate the specific characteristics of PTCL, a direct comparison of PTCL with a group of diffuse B-cell lymphomas (DBCL) was performed. Between June 1983 and December 1987, we studied 114 adults with NHL, using a battery of immunophenotyping markers. Adult T-cell leukemia/lymphoma, lymphoblastic lymphoma, mycosis fungoides/Sézary syndrome, follicular lymphoma, well-differentiated lymphocytic lymphoma, and true histiocytic lymphoma were excluded from this study since these are distinct clinicopathologic entities with well-recognized immunophenotypes. Of the remaining 75 patients, 70 who had adequate clinical information were analyzed, and of these, 34 were PTCL and 36 were DBCL. Classified according to the National Cancer Institute (NCI) Working Formulation (WF), 68% of PTCL and 31% of DBCL were high-grade lymphomas. Clinical and laboratory features were similar, except PTCL had a characteristic skin involvement and tended to present in more advanced stages with more constitutional symptoms. Induction chemotherapy was homogeneous in both groups, and complete remission rates were 62% for PTCL and 67% for DBCL. Patients with DBCL had a better overall survival than patients with PTCL, but the survival benefit disappeared after patients were stratified according to intermediate- or high-grade lymphoma. A subgroup of PTCL patients who had received less intensive induction chemotherapy was found to have a very unfavorable outcome. We conclude that (1) PTCL follows the general grading concept proposed in WF classification; (2) within a given intermediate or high grade, PTCL and DBCL respond comparably to treatment; (3) the intensity of induction chemotherapy has a crucial impact on the outcome of PTCL patients; and (4) with a few exceptions, the clinical and laboratory features of PTCL and DBCL are comparable.

scholarly journals Hodgkin's disease with a B-cell phenotype often shows a VDJ rearrangement and somatic mutations in the VH genes

Blood ◽  
1994 ◽  
Vol 84 (3) ◽  
pp. 708-715 ◽  
Author(s):  
J Tamaru ◽  
M Hummel ◽  
M Zemlin ◽  
B Kalvelage ◽  
H Stein
Keyword(s):  
T Cell ◽  
B Cells ◽  
B Cell ◽  
Hodgkin's Disease ◽  
Somatic Mutations ◽  
Hodgkin’S Disease ◽  
Receptor Gene ◽  
High Sensitivity ◽  
Cell Phenotype ◽  
Igh Genes

Abstract The nature of Hodgkin and Reed-Sternberg (HRS) cells remains in question. Immunophenotypic studies favor a relation to the lymphoid lineage with the existence of B- and T-cell types. However, studies on the detection of antigen (Ag) receptor gene rearrangements provided inconsistent results. They concur in that rearranged Ig and T-cell receptor (TCR) genes are not demonstrable in most Hodgkin's disease (HD) cases. To clarify whether this is because of the insensitivity of the method of detection or a real absence of clonal Ig heavy chain (IgH) rearrangements, a polymerase chain reaction (PCR) method with high sensitivity was applied, allowing the detection of less than 50 cells with clonally rearranged IgH genes in a mixture of 100,000 germline or individually rearranged cells. In 67 cases of HD, most of those (67%) with B-Ag+ HRS cells express clonal VDJ rearrangements of the IgH gene. No cases with T-cell Ag+ HRS cells harbored detectable clonal VDJ rearrangements. Of 10 sequenced rearranged IgH genes, the VH segment of six contained considerable somatic mutations. These results suggest that the demonstrated VDJ rearrangements stem from the HRS cells themselves and that the HRS cells of cases with rearranged IgH genes are B-cell related and correspond in their differentiation stage either to naive pregerminal center B cells or (more commonly) to germinal center/postgerminal center-derived memory B cells.

Cutaneous lymphomas other than mycosis fungoides: follow-up study of 52 patients.

1991 ◽  
Vol 9 (11) ◽  
pp. 1994-2001 ◽  
Author(s):  
P Joly ◽  
F Charlotte ◽  
M Leibowitch ◽  
C Haioun ◽  
J Wechsler ◽  
...  
Keyword(s):  
T Cell ◽  
B Cell ◽  
Mycosis Fungoides ◽  
Stage Iv ◽  
Disease Stage ◽  
Cell Phenotype ◽  
Low Grade ◽  
Cutaneous Lymphomas ◽  
Radiotherapy Alone

Cutaneous lymphomas other than mycosis fungoides (MF) represent a rare and heterogeneous group of lymphomas. Their clinical behavior remains largely unknown. In this study, the clinical and immunohistologic characteristics and follow-up data of 52 well-documented cases of cutaneous lymphomas other than MF, presenting with initial cutaneous lesions, were reviewed. Twenty-seven patients presented with skin disease alone (stage IE), and 25 patients had concurrent cutaneous and extracutaneous disease (stage IV). The tumors were grouped into high-grade lymphomas (HGLs; 21%), intermediate-grade lymphomas (IGLs; 58%), and low-grade lymphomas (LGLs; 21%). A B-cell phenotype was most often expressed by cutaneous lymphomas (73%), particularly by stage IE lymphomas (85%). Among 13 cases of T-cell lymphomas, loss of one of the pan-T-cell antigens was detected in all cases but one. The clinical course of cutaneous lymphoma was closely dependent on stage and histologic subtype but not on T-cell or B-cell phenotype. Of 20 patients with stage IV HGL or IGL, 13 were treated by polychemotherapy with curative potential. Their median survival was 37 months. Fourteen patients with stage IE HGL or IGL were treated by radiotherapy alone. Nine patients (69%) relapsed within 2 years posttreatment. Seven of them relapsed in the skin outside the initial site involved, suggesting that radiotherapy alone is not an adequate treatment for these patients. Preliminary results concerning seven other patients with stage IE IGL or HGL treated by an initial third-generation polychemotherapy regimen are presented.

scholarly journals CAR-T Cell Therapy

2020 ◽  
Vol 21 (12) ◽  
pp. 4303
Author(s):  
Aamir Ahmad
Keyword(s):  
Acute Lymphoblastic Leukemia ◽  
T Cell ◽  
Cell Therapy ◽  
B Cell ◽  
Hematological Malignancies ◽  
Lymphoblastic Leukemia ◽  
T Cell Therapy ◽  
Car T Cell Therapy ◽  
Car T ◽  
Large B Cell

CAR-T therapy has revolutionized the treatment of select hematological malignancies, namely, acute lymphoblastic leukemia and large B-cell lymphomas [...]

scholarly journals The expression of the Hodgkin's disease associated antigen Ki-1 in reactive and neoplastic lymphoid tissue: evidence that Reed-Sternberg cells and histiocytic malignancies are derived from activated lymphoid cells

Blood ◽  
1985 ◽  
Vol 66 (4) ◽  
pp. 848-858 ◽  
Author(s):  
H Stein ◽  
DY Mason ◽  
J Gerdes ◽  
N O'Connor ◽  
J Wainscoat ◽  
...  
Keyword(s):  
T Cell ◽  
B Cell ◽  
Hodgkin's Disease ◽  
Hodgkin’S Disease ◽  
Lymphoid Cells ◽  
Cell Phenotype ◽  
Cell Type ◽  
Lymphomatoid Papulosis ◽  
T Cell Lymphomas ◽  
Immunoblastic Lymphadenopathy

Abstract Ki-1 is a monoclonal antibody (raised against a Hodgkin's disease- derived cell line) that, in biopsy tissue affected by Hodgkin's disease, reacts selectively with Reed-Sternberg cells. The expression of Ki-1 antigen has been analyzed by immunocytochemical techniques in a wide range of human tissue and cell samples, including fetal tissue, malignant lymphomas (290 cases), and mitogen- and virus-transformed peripheral blood lymphocytes. The antigen was detectable on a variable proportion of cells in all cases of lymphomatoid papulosis and angio- immunoblastic lymphadenopathy and in 28% of the cases of peripheral T cell lymphomas (including lympho-epithelioid lymphomas). It was also expressed (more strongly) on tumor cells in 45 cases of diffuse large- cell lymphoma, most of which had originally been diagnosed as malignant histiocytosis or anaplastic carcinoma, because of their bizarre morphology. However, all of these cases lacked macrophage and epithelial antigens. Thirty-five cases expressed T cell-related antigens (associated in nine cases with the coexpression of B cell- related antigens), seven bore B cell-related antigens alone, and three were devoid of T and B cell markers. DNA hybridization with a JH specific probe showed a germline configuration in 11 cases of T cell phenotype, in two cases lacking T and B cell antigens, and in one case of mixed T/B phenotype, while rearrangement was found in two cases of clear B cell type and in one mixed T/B case. Expression of the Ki-1 antigen could be induced, together with interleukin 2 (IL 2) receptor, on normal lymphoid cells of both T and B cell type by exposure to phytohemagglutinin, human T leukemia viruses, Epstein-Barr virus, or Staphylococcus aureus. The results obtained indicate that Ki-1 antigen is an inducible lymphoid-associated molecule that identifies a group of hitherto poorly characterized normal and neoplastic large lymphoid cells. Tumors comprised solely of these cells show both morphological and immunological similarities to the neoplastic cells in Hodgkin's disease. This suggests that both disorders represent the neoplastic proliferation of activated lymphoid cells of either T cell or, less commonly, B cell origin. Disorders in which only a minority of cells express Ki-1 antigen (lymphomatoid papulosis, angio-immunoblastic lymphadenopathy, and certain T cell lymphomas) probably represent lesions in which only some of the abnormal cells have transformed into an “activation state.” In direct support of this view is the finding that the Ki-1 expression in these lesions is accompanied by the expression of HLA-DR and IL 2 receptors.

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