Primary lymphoplasmacytoid lymphoma of the trachea with immunoglobulin G paraprotein

A primary tracheal lymphoma with immunoglobulin G (IgG)-associated monoclonal serum paraprotein treated with surgery and chemotherapy is reported. As far as we know this is the first lymphoplasmacytoid lymphoma reported in the tracheobronchial tree and the first with a serum and tissue IgG monoclonal paraprotein. Differential diagnosis must be made essentially with extramedullary plasmacytoma and mucosa-associated lymphoid tissue lymphoma. CD-45RB strong positivity and the absence of lymphoepithelial lesions may help to differentiate lymphoplasmacytoid lymphoma from them. We expand the spectrum of lymphoid lesions with plasmacytoid features that can occur in the tracheobronchial tract.     

Impact of hepatitis C virus infection on clinical features, quality of life and survival of patients with lymphoplasmacytoid lymphoma/immunocytoma

Background: The non-Hodgkins lymphoma (NHL) subgroup most frequentlyassociated with hepatitis C virus (HCV) infection is the lymphoplasmacytoidlymphoma/immunocytoma (Lp-Ic). We have assessed the impact of the infectionon the clinical features, quality of life and survival of HCV+ve Lp-Icpatients as compared to its impact in HCV–ve patients.Patients and methods: Seventy patients with Lp-Ic consecutively observedover a six-year period were studied. Clinical, virological andhistopathological features were recorded at diagnosis. Quality of life wasassessed using a scoring system including disease-related symptoms,performance status, working ability, hospital admissions and therapiesrequired.Results: Eighteen patients (26%) with HCV infection wereidentified. Significant differences between those patients and theHCV–ve group included number of symptomatic patients, Hb levels, serumprotein levels, entity of the IgM monoclonal component, number of patientswith cryoglobulins and with organ (liver, kidney) involvement, and entityand pattern of bone marrow infiltration. Survival rates were similar (P =0.8383), but the quality-of-life score was significantly worse for theHCV+ve patients (P = 0.002). All anti-HCV Ab+ve patients tested positive forHCV RNA; genotype 2ac was detected in a significant proportion of cases.Conclusions: This study confirms that HCV infection is present in aboutone-third of patients with Lp-Ic. HCV infection does not seem to affect theoverall survival of patients with Lp-Ic, but it affects the clinicalexpression of the disease, so that the overall quality of life of HCV+vepatients is significantly worse.     

Monoclonal antibodies recognizing CD5, CD10 and CD23 in formalin-fixed, paraffin-embedded tissue: production and assessment of their value in the diagnosis of small B-cell lymphoma

AIMS: Assessment of the expression of antigens CD5, CD10 and CD23 can be of value in the differential diagnosis of small B-cell lymphoma. Correct subclassification is important since optimal treatment regimes differ between the subtypes. The aim of this study was to generate monoclonal antibodies recognizing these antigens in paraffin-embedded tissue and to assess their efficacy using a panel of cases of small B-cell lymphoma of various subtypes. METHODS AND RESULTS: For each antibody synthetic recombinant protein and conventional murine hybridoma technology was employed. Monoclonal antibodies effective in formalin-fixed, paraffin-embedded tissue were successfully generated, designated NCL-CD5-4C7, NCL-CD10-270 and NCL-CD23-1B12, respectively. A series of 58 cases of small B-cell lymphoma including examples of each subtype (lymphocytic, follicle centre cell, mantle cell, marginal zone and lymphoplasmacytoid) was assembled and immunostaining for the respective antigens carried out using the monoclonal antibodies produced. Our results indicate that the antibodies are specific for their respective antigens and give the predicted phenotypic profile in the small B-cell lymphoma subtypes. CONCLUSIONS: These novel monoclonal antibodies may be of value in routine diagnostic practice.     

Distribution of histologic subtypes and sex ratio in various primary sites of lymphocytic lymphoma.

To examine the distribution of histologic subtypes and sex ratio in each primary site of lymphoma, 1,169 histologically proven cases of lymphocytic lymphoma were analyzed. The location of tumor was nodal in 615(53%) and extranodal in 517(44%), patients with the gastrointestinal tract being the most common. The incidence was predominantly in males for all histologic types and in nodal and extranodal sites, except for a predominance of females in extranodal lymphoplasmacytic(Lp-cytic), lymphoplasmacytoid(Lp-cytoid) tumors. Frequency of the Lp-cytic/Lp-cytoid type among all types of lymphoma in females was about 2.7 times more frequent in extranodal than in nodal sites. The most striking example was thyroid lymphoma in which the frequency of Lp-cytic/Lp-cytoid type was 36% in female and 0% in male patients. Including this type of lymphoma, frequency of low grade lymphoma in females was higher in extranodal sites than in nodal sites.     

Gewebsmastzellenzahl bei Immunocytom und chronischer lymphatischer LeukÄmie

Summary The amount and distribution of tissue mast cells in the three subtypes of immunocytoma (IC) were studied in lymph nodes of 58 cases and compared with the findings on 34 cases of chronic lymphocytic leukemia (CLL). There were significantly more mast cells in the lymphoplasmacytic and lymphoplasmacytoid subtypes of IC than in CLL. The median mast cell count for the polymorphic subtype of IC was also greater than that for CLL; however, this difference was not statistically significant. Tissue mast cells were diffusely distributed in the lymph nodes in IC, whereas they were chiefly located in the sinus in CLL. Moreover, the cells themselves and their granules were generally larger in IC. Increase in the number and altered distribution of the tissue mast cells in histological sections are therefore diagnostic aids for distinguishing IC from CLL.

Herrn Dr. habil. Dr. rer. nat. L. Sachs (Abteilung Medizinische Statistik und Dokumentation der Christian-Albrechts-UniversitÄt Kiel) sind wir für seine RatschlÄge in der statistischen Analyse zu gro\em Dank verpflichtet.     

Lymphoid lesions of the conjunctiva: relation of histopathology to clinical outcome.

A retrospective clinicopathologic study of 40 patients with lymphoid lesions of the conjunctiva demonstrated the validity of current histologic criteria in predicting clinical outcome. Overall histologic architecture as well as cytologic detail must be used to differentiate benign reactive lymphoid hyperplasia from lymphoma. Lesions verified clinically as being malignant had obvious malignant cytologic features. Clinical signs of surface follicularity, multifocality, and minimal elevation suggest benignancy. All the benign lesions, on histopathologic examination, were either follicular in architecture or composed of mature lymphocytes, and were generally restricted to the substantia propria. Bilaterality and clinical recurrence do not necessarily imply a malignant disease.     

41例淋巴浆细胞样淋巴瘤治疗与预后分析

目的：探讨淋巴浆细胞样淋巴瘤的预后，以及治疗对该病预后的影响。方法：对４１例淋巴浆细胞样淋巴瘤患者均进行了较积极的治疗及长期随访，并经统计学处理。结果：全组中位生存期３９．４个月。导致生存期缩短的主要原因是就诊时临床分期已达Ⅲ、Ⅳ期（Ｐ＜０．０１）；具有Ｂ症状（Ｐ＜０．０５）；骨髓及结外受累（Ｐ＜０．０１）。综合治疗的无瘤生存期及１年生存率高于单纯化疗（Ｐ＜０．０５），但二者生存期无显著差别（Ｐ＞０．０５）。结论：对淋巴浆细胞样淋巴瘤不应过于积极治疗，应静观其变化，适时给予必要的干预，治疗主要目的应是改善患者的生存质量。     

IgM AL amyloidosis due to B cell lymphoproliferative disorder: efficacy of high-dose melphalan followed by autologous stem cell transplantation

This report concerns a patient with IgM AL amyloidosis due to a B cell lymphoproliferative disorder who was successfully treated with VAD and subsequent high-dose melphalan followed by autologous stem cell support. After this chemotherapeutic regimen, the patient showed complete hematological remission and improvement in nephrotic syndrome. These findings suggest that high-dose melphalan may also be effective for lymphoplasmacytoid cells producing monoclonal IgM which are phenotypically distinct from plasma cells. Myeloablative therapies, such as high-dose melphalan, should definitely be considered as a treatment option for AL amyloidosis, irrespective of the type of precursor immunoglobulin.     

Beneficial effects of rituximab on primary cold agglutinin disease refractory to conventional therapy

A case is reported of lymphoplasmacytoid lymphoma (LPL) associated with a monoclonal immunoglobulin (Ig) M and cold agglutinin disease (CAD) that was successfully treated with rituximab. A 52-yr-old male was admitted with a direct antiglobulin test positive haemolytic anaemia and thrombocytopenia associated with monoclonal IgM. Bone marrow examinations disclosed the marked infiltration of medium-sized lymphoma cells with plasmacytoid differentiation that indicated non-Hodgkin's lymphoma of B-cell origin (LPL). Prednisolone and combination chemotherapy were temporarily effective for both anaemia and thrombocytopenia, although these strategies became refractory and bone marrow lymphoplasmacytosis persisted. CAD ameliorated, and the serum level of IgM decreased in association with the disappearance of lymphoma cells and clonal rearrangement of the Ig heavy chains in the bone marrow after treatment with rituximab. Rituximab played a significant role in the treatment of refractory CAD associated with LPL.     

IgG-secreting lymphoplasmacytoid leukaemia: a B-cell disorder with extensively mutated VH genes undergoing Ig isotype-switching frequently associated with trisomy 12

We investigated 16 patients with elevated serum monoclonal IgG and a leukaemic B-cell lymphocytic disorder different from multiple myeloma. Their clinical history was that of a non-aggressive disease with dominant splenomegaly and long survival. Whereas abnormal blood and bone marrow cells were predominantly small lymphocytes with a few lymphoplasmacytoid cells, histopathological features included a lymphoplasmacytic infiltrate in eight cases. Most frequently, abnormal blood cells displayed a CD19+CD5-CD23+/- immunophenotype different from that of chronic lymphocytic leukaemia, except in two cases with a CD19+CD5+CD23+ phenotype. Interestingly, a coexistent serum monoclonal IgM and/or surface IgMG+ with identical light chain was identified in 10 patients, whereas in the remaining six patients only IgG expression was determined. VH gene analysis was performed in eight patients to investigate the clonal origins of tumour cells. All cases utilized the VH3 family, with evidence of extensive somatic mutations and intraclonal homogeneity in all cases. VH gene analysis indicated a clonal relationship between cells expressing IgM and IgG, with one case being biclonal. Cytogenetic evaluation showed a high incidence of trisomy 12 (60%) and 13q14 deletion (40%). In conclusion, we have described an unusual subset of low-grade lymphoma with high-serum IgG and frequent lymphoplasmacytoid features in which tumour cells derive from post-follicular memory B cells undergoing isotype switching with some cases arrested at both the IgM and IgG stage and others as IgG-positive cells only.