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Recurrence of nodal diffuse large B-cell lymphoma as intravascular large B-cell lymphoma: is an intravascular component at initial diagnosis predictive?

We report a case of a 59-year-old man who first presented with a nodal diffuse large B-cell lymphoma that later relapsed as an intravascular large B-cell lymphoma. In the initial biopsy specimen, a few intranodal small vessels that contained large lymphoma cells were noted. After 8 months of multiagent chemotherapy, clinical remission was attained. Two years after the initial diagnosis of nodal diffuse large B-cell lymphoma, the patient presented with a rapid onset of multiorgan failure, which at autopsy was shown to be due to intravascular large B-cell lymphoma. Molecular genetic studies showed that these 2 lymphomas had immunoglobulin heavy-chain gene rearrangements that were of identical size, suggesting that they were derived from the same clone. To our knowledge, this is the first report of a nodal large B-cell lymphoma that relapsed as an intravascular large B-cell lymphoma. Although this report is of only a single case, the presence of a relatively inconspicuous intravascular component in an otherwise typical nodal large B-cell lymphoma may be predictive and could affect clinical decisions regarding diagnosis, monitoring, and prognosis of such lymphomas.

Fatal Outcome↗

Mantle cell lymphoma disguised as marginal zone lymphoma.

We report a case of mantle cell lymphoma histologically indistinguishable from marginal zone lymphoma. An 83-year-old man presented with a 9.0-cm, slowly enlarging axillary mass. Microscopically, the neoplastic process was largely interfollicular, surrounding residual follicular centers, some of which had discernible small lymphocyte mantles. Overall, the morphologic pattern was highly suggestive of marginal zone lymphoma. However, flow cytometric and immunohistochemical results, including cyclin D1 positivity, revealed an immunophenotype that fit with mantle cell lymphoma. The differential diagnosis of mantle cell lymphoma is broad, and it is well known that mantle cell lymphoma can assume a number of histologic appearances, including, infrequently, that of more indolent B-cell non-Hodgkin lymphomas. Although not pathognomonic, cyclin D1 positivity is highly specific for mantle cell lymphoma and is key in distinguishing these clinically dissimilar malignant lymphomas. In recent years, detection of cyclin D1 has expanded the recognizable histologic spectrum of mantle cell lymphoma.

Aged↗

[Is it possible to individualize microvillous large-cell lymphoma among sinusoid lymphomas?].

Microvillous non-hodgkin lymphomas are large cell malignant lymphomas with a sinus growth pattern and the presence of cytoplasmic processes detected by electron microscopy. Microvillous non-hodgkin lymphomas appear to be of B cell lineage. Neoplasms considered in the differential diagnosis include anaplastic large cell Ki-1 lymphomas, malignant histiocytosis and metastatic carcinoma or malignant melanoma. A panel of markers are usually sufficient to recognize all of these neoplasms except microvillous lymphomas. We report a case of microvillous non-hodgkin lymphoma characterized by a histologic pattern mimicking those of anaplastic large cell Ki-1 lymphoma. However, immunohistochemistry study failed to demonstrate presence of activation antigen such as Ki-1, EMA, interleukin 2 receptor. Ultrastructural study showed that neoplastic cells exhibited filliform cytoplasmic processes. This report raise the possibility of an overlap between microvillous non-hodgkin lymphomas and anaplastic large cell Ki-1 lymphomas of B-cell lineage. It must be emphasized that, initially, these two kinds of lymphomas were defined with different morphologic technologies.

Biomarkers, Tumor↗

Bcl-2 gene rearrangements in primary B-cell lymphoma of the gastrointestinal tract reveal follicular lymphoma as a subtype.

The principal objective of this study was to investigate whether follicular center cell lymphomas occur among B-cell lymphoma of mucosa-associated lymphoid tissue (MALT). We used a molecular genetic/immunohistochemical approach and analysed 21 cases with the primary site in the gastrointestinal tract. Only two bcl-2 gene rearrangements were detected in our series and were found in two out of seven lymphomas with a nodular growth pattern. A chromosomal translocation t(14;18) was demonstrated by comigration of rearranged bcl-2 and JH sequences in one of these two cases. Additionally, both lymphomas showed bcl-2 protein positive neoplastic follicles, CD10 expression, and lack of vimentin. Therefore, these two cases were defined as follicular lymphomas. In contrast to the two follicular lymphomas of MALT, three other, nodular growing, bcl-2 protein positive lymphomas were found to have no bcl-2 gene rearrangements, to be CD10 negative and to express vimentin. These three lymphomas might be composed of neoplastic extrafollicular cells which secondarily invaded reactive follicles. We conclude that the presence of bcl-2 protein positive follicles is consistent with both a follicular and extrafollicular origin of a B lymphoma of MALT. However, the detection of a bcl-2 gene rearrangement is the most valuable criterion in such a situation, and additional immunophenotypic criteria, such as CD10 expression and lack of vimentin within the neoplastic population, further substantiate the diagnosis of a follicular lymphoma in MALT.

Adult↗

Terminal deoxynucleotidyl transferase staining of malignant lymphomas in paraffin sections: a useful method for the diagnosis of lymphoblastic lymphoma.

Terminal deoxynucleotidyl transferase (TdT) is a DNA polymerase located in the cell nucleus which catalyses the polymerization of deoxynucleotides at the 3'hydroxyl ends of oligo- or polydeoxynucleotide initiators without a template. TdT is known as a useful marker for the diagnosis of acute lymphoblastic leukaemia/lymphoma, but its detection usually requires fresh tissue specimens or cell suspensions, using either an enzyme analysis or immuno-fluorescence or -peroxidase staining. Until the recent development of the use of microwave-treated paraffin sections for immunoperoxidase staining, detection of TdT in paraffin sections required rather complicated processes. This new simple technique was applied to paraffin sections from the tumour tissue specimens of 16 patients with lymphoblastic lymphoma and of seven patients with non-endemic Burkitt's lymphoma, which is sometimes difficult to differentiate from lymphoblastic lymphoma because of their similar clinicopathological characteristics. In addition, as a control, ten cases each were examined of adult T-cell leukaemia/lymphoma (ATLL) and angioimmunoblastic lymphoma (AILD), which are both peripheral T-cell lymphomas. The tumour cells from 15 of the 16 (94 per cent) patients with lymphoblastic lymphoma were found to be TdT-positive. The specificity of the anti-TdT antibody used was confirmed by immunoblot and the specific 60 kD band was detected only in a specimen of lymphoblastic lymphoma. These results show that the immunostaining of TdT on paraffin-embedded sections is a useful method for differentiating lymphoblastic lymphoma from other lymphomas. This method is applicable to a routine diagnostic service.

Adolescent↗

Exclusive detection of the t(11;18)(q21;q21) in extranodal marginal zone B cell lymphomas (MZBL) of MALT type in contrast to other MZBL and extranodal large B cell lymphomas.

Extranodal mucosa-associated lymphoid tissue (MALT)-type lymphomas and nodal and splenic marginal zone B cell lymphomas (MZBL) share morphological and immunophenotypic features with marginal zone B cells of reactive lymphoid tissues. Although displaying a similar immunophenotype, recent investigations suggest fundamental genetic differences among these subgroups. To determine the prevalence of the t(11;18) in a larger series of MALT-type lymphomas and to investigate a possible occurrence in other lymphomas, we screened 106 non-Hodgkin's lymphomas (NHL) by interphase cytogenetics using yeast artificial chromosome (YAC) probes flanking the breakpoint at 11q21. A signal constellation indicating a disruption in 11q21 and thus pointing to the presence of the t(11;18) was observed in 9 of 33 (27%) low-grade lymphomas of MALT type. The complete absence of t(11;18)-positive cells in 32 primary and secondary extranodal high-grade lymphomas suggests that low-grade lymphomas of MALT type characterized by the t(11;18) are unlikely to transform into high-grade tumors. The absence of tumor cells carrying the t(11;18) in nodal MZBL challenges the assumption that most, if not all, of these tumors represent the nodal manifestation of a so far undetected low-grade lymphoma of MALT type. The t(11;18) was not detected in a single case of 29 splenic MZBL investigated. This observation strengthens the view that splenic MZBL are biologically different from extranodal MZBL of MALT type.

Chromosome Banding↗

CXCR3-positive B cells found at elevated frequency in the peripheral blood of patients with MALT lymphoma are attracted by MIG and belong to the lymphoma clone.

Chemokine receptors mediate the migration of lymphocytes through binding of their ligands. CXCR3 is expressed in Th1 T cells; however, CXCR3 was recently reported in B-cell mucosa-associated lymphoid tissue (MALT)-type lymphoma and splenic marginal zone lymphoma. To investigate whether CXCR3-positive B lymphocytes in peripheral blood (PB) migrate to MALT and spleen, and whether the lymphoma clone is present in PB, we studied 16 cases of MALT lymphoma. In MALT cases, CXCR3-positive B lymphocytes in PB could migrate to MIG, the CXCR3 ligand. Immunohistochemical analysis showed that MALT lymphoma cells expressed CXCR3, whereas epithelial glands and/or stromal cells expressed MIG. In the PCR analysis for VH gene rearrangements, MALT lymphoma showed monoclonal or oligoclonal bands. In addition, in 8 of 16 MALT cases, the VH gene rearrangement of MALT lymphoma had the same bands as the CXCR3-positive B lymphocytes in PB. In 4 cases, the same clones of DNA sequences were confirmed in MALT lymphoma and CXCR3-positive B lymphocytes of PB. The findings support the theory that CXCR3-positive B lymphocytes in PB of MALT patients belong to the lymphoma clone and migrate to MIG-expressing mucosa-associated lymphoid tissue. It seemed to be associated with the dissemination of MALT lymphoma.

Adult↗

Prelymphoma, early lymphoma, and manifest lymphoma in immunosialadenitis (Sjögren's syndrome)--a model of lymphomagenesis.

The development of malignant lymphoma in myoepithelial sialadenitis with and without Sjögren's syndrome was investigated. At first, prelymphomatous proliferation areas showing a polytypic immunoglobulin (Ig) pattern were seen. These transformed into malignant lymphoma with a monotypic Ig pattern, at first in small, circumscribed proliferation areas ("early lymphoma") and later in large, confluent proliferation area ("manifest lymphoma"). The lymphomas were classified as LP immunocytoma. In some cases they transformed into high-grade malignant lymphomas of the same category, namely, B-immunoblastic lymphoma. The same types of lymphoma have been found in NZB mice and chronic graft-versus-host reactions. "Primary" malignant lymphomas of salivary glands that did not show myoepithelial sialadenitis were also studied. These lymphomas were mostly germinal center cell tumors and probably developed primarily in lymph nodes within parotid glands in most, if not all, cases.

Cell Division↗

[Glomerulonephritis and malignant lymphoma. Mostly men with low-grade lymphoma with various forms of glomerulonephritis].

BACKGROUND: The association of non-Hodgkin's lymphoma with different types of glomerulonephritis is well-known for many years. Whereas in Hodgkin's disease, minimal change glomerulonephritis is mainly observed, in non-Hodgkin's lymphoma various forms of glomerulonephritis are found. PATIENTS AND RESULTS: We describe 3 cases of non-Hodgkin's lymphoma, which were associated with glomerulonephritis. Two cases of glomerulonephritis showed nephrotic syndromes, which improved by medicinal treatment. In 2 cases glomerulonephritis and non-Hodgkin's lymphoma developed simultaneously. In a third lymphoma was followed by glomerulonephritis after 3 years. CONCLUSION: Although the pathogenetic relationship between non-Hodgkin's lymphoma and glomerulonephritis is not fully understood, the high number of reported cases yield interdependence. Lymphoma associated glomerulonephritis is found more often in men than in women. Low-grade non-Hodgkin's lymphomas--especially chronic lymphatic leukemias--seem to develop glomerulonephritis more frequently than high-grade. The majority of non-Hodgkin's lymphoma do not show any predisposition to a special type of glomerulonephritis, only in cutaneous lymphoma IgA-nephropathy predominates.

Aged↗

The FOXP1 transcription factor is expressed in the majority of follicular lymphomas but is rarely expressed in classical and lymphocyte predominant Hodgkin's lymphoma.

The expression of the FOXP1 forkhead/winged helix transcription factor has been investigated in normal and neoplastic lymphoid tissues using the FOXP1-specific monoclonal antibody, JC12. Using single and double immunoenzymatic staining, FOXP1 expression has been studied in a series of classical and lymphocyte predominant Hodgkin's lymphomas, follicle centre lymphomas and Hodgkin's lymphoma-derived cell lines. Neoplastic cells in the majority of classical and lymphocyte predominant Hodgkin's lymphoma were FOXP1-negative. In two cases of classical Hodgkin's lymphoma, the tumour cells showed mislocalisation of FOXP1 to the cytoplasm, while in one case of lymphocyte predominant Hodgkin's lymphoma, and in the Hodgkin's lymphoma cell line KMH2, scattered tumour cells showed nuclear expression of FOXP1. In contrast, the tumour cells in the majority of follicle centre lymphomas showed strong nuclear FOXP1 reactivity. Double labelling studies of lymphoid tissue indicated that a variable proportion of CD20-positive germinal centre B cells express FOXP1 while the vast majority of CD30-positive cells are FOXP1-negative. The heterogeneity of FOXP1 expression in germinal centre-derived lymphomas, may have more to do with the transforming events underlying these distinct types of lymphoma than with their common origin.

Cell Line, Tumor↗

Paraproteins and primary lymphoma in SJL mice. II. Primary lymphomas do not produce paraprotein.

The incidences of Ig paraprotein (PP) and reticulum cell sarcomas (lymphomas) were studied in a group of 75 SJL mice, 9-11 months of age. PP and lymphoma were observed in 52% of the mice. PP alone was observed in an additional 27% and lymphoma alone in an additional 11% of mice. In attempts to establish a causal relationship between lymphomas and PP, three approaches were used: (a) Serum PP levels were followed during lymphoma growth in primary lymphoma bearing mice and found to decrease rather than to increase. (b) Recipients of primary transplants were examined for appearance of PP-related idiotypes (Id) in their sera and for lymphoma growth. The Id appeared early (Day 10-11) and then disappeared, while lymphoma growth usually was detectable by greater than or equal to 1 month. (c) One of the primary lymphomas was propagated as a tissue culture line and found not to produce any PP or intact Ig. It is concluded that the PP of SJL mice are not produced by their lymphomas. Other possible relationships are discussed, including the role of the PP as a symptom of a prelymphomatous stage that develops in a very high percentage of SJL mice.

Animals↗

Composite low grade B-cell lymphomas with two immunophenotypically distinct cell populations are true biclonal lymphomas. A molecular analysis using laser capture microdissection.

Low grade B-cell lymphomas comprise several well defined, clinically and immunophenotypically distinct disease entities. Composite lymphomas showing phenotypic characteristics of more than one of these tumor subtypes in the same site are rare, and both common and separate clonal origins of the two tumor parts have been reported for cases studied by molecular methods. We describe the detailed immunohistochemical and molecular findings in three cases with features of composite low grade B-cell non-Hodgkin's lymphoma (B-NHL). All three neoplasms contained morphologically distinct but interwoven compartments of different cell types, which exhibited discordant expression of several markers, including CD5, CD10, CD43, and cyclin D1. According to their morphology and phenotypes, they were classified as mantle cell lymphoma and follicular lymphoma (Case 1), follicular lymphoma and small lymphocytic lymphoma (Case 2), and mantle cell lymphoma and chronic lymphocytic leukemia/small lymphocytic lymphoma (Case 3). PCR analysis of DNA obtained from whole tissue sections failed to reveal evidence for biclonality in any of the cases. We therefore isolated cell populations with different antigen expression patterns by laser capture microdissection and analyzed them by polymerase chain reaction amplification and sequencing of clonal immunoglobulin heavy chain gene rearrangements and oncogene rearrangements. Sequence analysis revealed unrelated clonal rearrangements in each of the two tumor parts in all three cases, suggesting distinct clonal origins. In addition, Case 1 showed a bcl-2 rearrangement present only in the follicular lymphoma part. Our findings suggest that low grade B-NHL with two distinct morphological and immunophenotypic patterns in the same anatomical site are frequently biclonal. This is in keeping with current classification schemes, which recognize subtypes of low grade B-NHL as separate disease entities. Furthermore, our analysis demonstrates the power of laser capture microdissection in revealing molecular microheterogeneity in complex neoplasms.

Aged↗

Clinical features of peripheral T-cell lymphomas in 78 patients diagnosed according to the Revised European-American lymphoma (REAL) classification.

The aim of this study was to analyse the clinical characteristics and prognostic factors of peripheral T-cell lymphomas (PTCLs) according to the Revised European-American Lymphoma (REAL) classification. From 1994 to 1999, 78 patients were diagnosed with PTCLs, excluding cutaneous T-cell lymphomas and T-cell chronic lymphocytic leukaemia. The distribution of the histological subgroups were: PTCL unspecified (PTCL-U), 40%; angiocentric lymphoma, 32%; anaplastic large cell lymphoma (ALCL), 17%; angioimmunoblastic T-cell lymphoma (AILD), 6%; intestinal T-cell lymphoma, 3%; and panniculitic T-cell lymphoma, 3%. Patients with angiocentric lymphoma presented with favourable prognostic factors, whereas those with AILD presented with unfavourable prognostic factors. Most patients were treated with doxorubicin-containing combination chemotherapy (with or without radiation therapy). The overall complete remission rate was 61.2% (95% Confidence Interval (CI): 48.5-72.8%) and the 5-year probability of failure-free survival was 33.5%. Median survival of all patients was 45 months (range 0-64+ months) and the 5-year probability of survival was 36.2%. In the multivariate analysis, only the International Prognosis Index (IPI) was an independent prognostic factor for overall survival (P<0.01). Taken together, the proportion of angiocentric lymphoma in this study was higher than that in the studies of Western countries. PTCL responds poorly to treatment with low survival rates and the IPI is a useful prognostic factor for PTCL.

Adolescent↗

Mantle cell lymphomas with clonal immunoglobulin V(H)3-21 gene rearrangements exhibit fewer genomic imbalances than mantle cell lymphomas utilizing other immunoglobulin V(H) genes.

A preferential use of one particular immunoglobulin variable heavy chain gene, V(H)3-21, has recently been reported in mantle cell lymphoma, where almost all of these V(H)3-21+ mantle cell lymphomas showed usage of the same light chain Vlambda gene (Vlambda3-19) and also had a tendency towards improved prognosis. These findings suggested that V(H)3-21+ mantle cell lymphomas constitute a distinct subgroup, possibly with antigen stimulation involved in disease pathogenesis. In this study, we applied the comparative genomic hybridization (CGH) method on 37 mantle cell lymphoma tumors in order to investigate if the V(H)3-21+ tumors are different at the genomic level. Interestingly, V(H)3-21+ mantle cell lymphomas (n=14) showed significantly fewer genomic aberrations (mean 2.4) compared to non-V(H)3-21 mantle cell lymphomas (n=23) (mean 4.9). The chromosomal aberrations identified in our study were generally in accordance with previous CGH studies of mantle cell lymphoma; the most frequent aberration was complete or partial loss of chromosome 13, followed by recurrent losses within 6q, 9p, 9q and 11q and frequent gains in 3q, 7p, 8q and 15q. Deletions within 8p and 9p as well as gains in 7p and 15q were found exclusively in the non-V(H)3-21-utilizing tumors. In summary, V(H)3-21+ mantle cell lymphomas demonstrated both a lower number and a different spectrum of genomic aberrations than mantle cell lymphoma in general, thus supporting the hypothesis that V(H)3-21+ mantle cell lymphomas constitute a new subgroup. The findings presented in this report may explain the tendency for a better clinical outcome for patients whose tumors utilize V(H)3-21.

Aged↗

Diffuse T-cell lymphoma preceded by nodular lymphoma.

A patient with a nodular lymphoma morphologically indistinguishable from follicular B-cell lymphoma subsequently developed a diffuse T-cell lymphoma. This T-cell lymphoma displayed characteristics analogous to mature T-lymphocytes, and typed as a suppressor/cytotoxic T-cell with the monoclonal antibodies. The light and electron microscopy morphology of the T-cell lymphoma was similar to that reported for node-based T-cell lymphomas. There was a polymorphous proliferation of variably transformed lymphocytes including immunoblasts. This case could represent either a nodular form of T-cell lymphoma converting to a diffuse T-cell lymphoma, or a follicular B-cell lymphoma preceding, and possibly inducing, a suppressor T-cell lymphoma.

Adult↗

Resistance to syngeneic lymphoma cells as a result of immunization with chemically modified allogeneic lymphoma cells in mice.

Immunization with trinitrophenyl (TNP)-derivatized allogeneic lymphoma cells resulted in significant immunity to poorly immunogenic syngeneic lymphoma cells. Neither TNP-treated nor X-irradiated syngeneic lymphoma cells were immunogenic under similar experimental conditions. Immunization with untreated allogeneic lymphoma cells produced only minimal levels of resistance to challenge with syngeneic lymphoma cells. The complete set of antigens responsible for the immunity was carried exclusively on transformed lymphocytes because allogeneic TNP-derivatized lymph node and thymus cells also did not immunize. The immunity was transferred to nonimmune inbred BALB/c and A/J mice by spleen cells from immune donors. The Winn assay was used to measure the antilymphoma immunity in vivo. When immune spleen cell-lymphoma mixtures were inoculated sc at a ratio of 1,000:1, nonimmune mice were completely protected. Reactivity of immune lymphocytes to syngeneic lymphoma cells was also demonstrated in vitro by the 51Cr-release method. Immunization with TNP-derivatized allogeneic lymphoma cells resulted in measurable immune resistance to inocula of viable syngeneic tumor cells in excess of 100 times the tumorigenic dose. Induction of immunity to syngeneic lymphoma cells strictly required that the immunizing cells be histoincompatible at the major H-2 locus and possess tumor-specific antigen(s). Maximum immune sensitivity was observed only after chemical modification of the immunizing allogeneic lymphoma cells.

Animals↗

Transformation of follicular lymphoma into CD30-large cell lymphoma with anaplastic cytologic features.

The natural history of follicular lymphoma is to accrue large cells and become diffuse, resulting in progression/transformation to a higher-grade lymphoma. Histologic transformation occurs in approximately 60% of patients. Most often, follicular lymphomas transform into diffuse large cell lymphoma, but transformation to lymphomas classified using the Working Formulation as diffuse mixed, large cell immunoblastic, or small noncleaved cell also have been reported. Evidence of transformation may be found over time in sequential biopsy specimens, or may coexist in the same biopsy specimen. Here, we describe six cases of follicular lymphoma, large cell in five cases and mixed in one case, that transformed into a diffuse or sinusoidal CD30 antigen-positive large cell lymphoma with anaplastic cytologic features. Both the follicular and diffuse/sinusoidal components were of B-cell lineage, positive for the CD20 antigen and negative for the CD3 and CD43 antigens. The neoplastic cells expressed monotypic immunoglobulin light chain in five cases, three kappa and two lambda. BCL-2 protein was positive in four tumors, in both the follicular and diffuse/sinusoidal components in three cases, and only in the latter component in one case. Using the polymerase chain reaction (PCR), three of six cases had monoclonal immunoglobulin heavy chain gene rearrangements. The t(14;18) was not amplified in any case. Using reverse transcriptase (RT)-PCR, the t(2;5) was amplified in one of four tumors. This report highlights the heterogeneity of B-lineage anaplastic large cell lymphomas and indicates the need to consider antecedent follicular lymphoma in any B-cell lymphoma with anaplastic cytologic features.

Adult↗

Pathologic and clinical features of 77 Hodgkin's lymphoma patients treated in a lymphoma protocol (LNH87): a GELA study.

Between 1987 and 1993, 77 of 2855 lymphomas included in the LNH87 protocol of the GELA as non-Hodgkin lymphoma (NHL) and reviewed by a panel of pathologists had a diagnosis changed to Hodgkin lymphoma (HL). Some of these lymphomas had been initially interpreted as anaplastic large-cell lymphoma Hodgkin-like (ALCL-HL subtype). The purpose of this study was to analyze the histologic pitfalls initially encountered, to define more clearly the diagnostic criteria of lymphomas placed in the gray zone around HL, and to follow the survival of these 77 patients affected with HL and initially treated with NHL regimens. The 77 cases of HL were reviewed by three hematopathologists and immunostained with a large panel of antibodies, including CD30, CD15, CD3, CD20, CD45, CD43, LMP-1, EMA, BNH-9, TiA1, and ALK1. Each case was classified according to the Lukes-Rye system and the British National Lymphoma Investigation (BNLI) grading. The initial clinical presentation of patients was analyzed, and the overall and event-free survival rates of the 77 patients were estimated. Among the 77 HLs, 46 were misinterpreted as NHL by primary individual pathologists (12 as ALCL, 8 as ALCL-HL, 12 as peripheral T-cell lymphoma (PTCL), 6 as B-cell lymphoma, and 8 as unclassifiable NHL). The other 31 cases had been first considered by the panel as consistent with ALCL-HL (n = 18) or with PTCL (n = 13) and were changed later in view of an immunophenotype concordant with HL. Fifty-five percent of the patients completed the full NHL treatment. The 5-year event-free and overall survival rates were 54% and 77%, respectively. The current results indicate that lymphomas initially called ALCL-HL should not be regarded as a variant of ALCL, but as HL. The clinical consequences of misdiagnoses seem to be a lower event-free survival rate compared with that of classical HL, probably because of more relapses of initially inappropriately treated HL.

Adolescent↗