[Hyperviscosity syndrome in patients with immunoproliferative disorder].
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Blood Viscosity↗
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The clinical spectrum of pure Bence Jones proteinuria. A study of 66 patients.
Sixty-six consecutive patients exhibiting isolated urinary excretion of monoclonal free light chains, i.e. Bence Jones protein (BJP), on screening investigation for serum and urine monoclonal immunoglobulins were studied in order to better define the spectrum of immunoproliferative disorders associated with such a protein abnormality. The typical plasma cell neoplasms accounted for only one third of the cases, multiple myeloma (MM) and systemic amyloidosis (AL) being diagnosed in 18% and 15% of the patients, respectively. Eighteen (27%) of the patients were recognized as having malignant nonHodgkin's lymphomas (NHL), 21 (32%) had chronic lymphocytic leukemia (CLL), and 2 (3%) had hairy cell leukemia (HCL). Three patients (5%) without apparent evidence of any malignant immunoproliferative disease were classified as having a monoclonal gammopathy of undetermined significance (MGUS). The greatest urinary concentrations of BJP were found in plasmacytic neoplasms, the daily excretion of MM patients being significantly higher than that of AL patients. Considerably lower BJP outputs were recorded in the other diseases, the lowest ones being associated with MGUS. NHL patients had a daily excretion four times higher as compared with that of CLL patients. The distribution of NHL by histologic type was: follicular center cell lymphomas (FCCL) 39%, small lymphocytic lymphoma (SLL) 33%, immunoblastic lymphoma (IBL) 17%, and plasmacytoid lymphocytic lymphoma (PLL) 11%. The highest BJP levels were found in PLL, and the lowest ones in FCCL. In CLL patients the amount of urinary BJP correlated significantly with the tumor load, as estimated by the number of enlarged lymphoid areas. The study suggests that detection and measurement of isolated urinary BJP may provide useful data for the clinical evaluation of a wide spectrum of immunoproliferative disorders.
Aged↗
A study of familial factors in Alzheimer's disease.
Data on the families of 74 probands with autopsy-proven Alzheimer's disease did not support the hypothesis, advanced by Heston and co-workers, of a familial association between Alzheimer's disease, Down's syndrome and immunoproliferative disorders. However, there are difficulties of interpreting negative conclusions in this type of study, particularly those resulting from small sample size and the impossibility of tracing all relatives; only the data for immunoproliferative disorders are incompatible with the hypothesis, those for Down's syndrome being too few to be informative. The incidence of presenile dementia among the first-degree relatives of probands was raised, as in many previous studies, and was consistent with a simple polygenic model. The mean parental age at birth of the probands was significantly raised by about 2 years (P divided by 0.01), but so also was that of their unaffected sibs, suggesting that the mechanism differs from that occurring in trisomy 21 and certain other aneuploidies.
Adolescent↗
Waldenström-like immunocytic lymphoma with IgG serum M component.
A case of immunoproliferative disorder with clinical features of Waldenström's disease but with an IgG-k instead of IgM serum M component is described. The lymphocyte population in the bone marrow, blood and lymph nodes was studied by rosette test, culture with PHA and immunofluorescence staining. Most of the bone marrow lymphocytes had membrane IgG. This case represents an intermediate form between myeloma and Waldenström's disease, thus supporting the unitarian concept of all the immunoproliferative disorders.
Fluorescent Antibody Technique↗
Human T4+ lymphocytes produce a phagocytosis-inducing factor (PIF) distinct from interferon-alpha and interferon-gamma.
Unstimulated peripheral blood mononuclear cells from patients with angiocentric T cell immunoproliferative disorders and concanavalin A-stimulated normal peripheral blood mononuclear cells secrete a phagocytosis-inducing factor (PIF) that induces a fivefold to 50-fold enhancement of phagocytosis of IgG-coated ox red blood cells by U937 cells. We investigated the identity, production, and mechanism of the action of PIF. PIF activity was demonstrated in supernatants from nine of 44 phytohemagglutinin-stimulated interleukin 2 (IL 2)-dependent T cell lines and clones derived from purified T4+ cells, but was not found in supernatants from 26 lines and clones derived from phytohemagglutinin-stimulated T8+ cells. In addition, PIF was produced by four of four antigen-specific T cell lines and clones after stimulation with the appropriate antigen and antigen-presenting cells, and by HUT-102, a human T cell lymphotropic virus type I-transformed T cell line. PIF from all of these sources caused significant inhibition of U937 proliferation. This proliferation-inhibiting activity co-purified with phagocytosis-enhancing activity in sizing procedures and isoelectric focusing, which yielded an estimated m.w. of 35,000 to 55,000 and an estimated isoelectric point of 5.0 to 6.0 for PIF. In contrast, IL 2, recombinant interferon-alpha, and recombinant interferon-gamma had no effect on phagocytosis by U937 cells, and antibodies to interferon-alpha and interferon-gamma did not block the phagocytosis-inducing activity of PIF-containing supernatants. PIF appears to be a distinct lymphokine produced by a subset of T4+ lymphocytes, possibly those that proliferate in response to antigen. PIF may be important in the induction of erythrophagocytosis, which is associated with certain T cell immunoproliferative disorders.
Antigens, Differentiation, T-Lymphocyte↗
Immunobeads test (antibody coated polyacrylamide beads) in the immunological characterization of lymphoproliferative disorders.
Three groups of patients with immunoproliferative disorders (15 multiple myeloma, 11 non-Hodgkin's lymphoma, 21 chronic lymphocytic leukemia) were studied by immunological characterization and compared to a group of 20 normal subjects (controls) using anti-immunoglobulin coated polyacrylamide beads (T-B Quantigen test, QT), erythrocyte rosettes (ER), surface immunoglobulin (SIg), and monoclonal antibodies for T and B cells (OKT3; OKT11; OKT8; OKT4; IaDR); null cells (NC) and double marker (DM) cells were also considered. The values for normal subjects for T-B, NC and DM cells were comparable. Results for the patient groups strikingly differed. There were progressively larger differences between the T and B percentages obtained with different techniques. The largest differences were seen in patients with chronic lymphocytic leukemia and the smallest in multiple myeloma patients; values were intermediate in non-Hodgkin lymphoma. The different findings were related to the number of DM cells (ER+, SIg+ QT+) and the different tests used. The importance of these findings in the diagnostic approach to lymphoproliferative disorders is discussed.
Acrylic Resins↗
The excretion of tryptophan metabolites in patients with different forms of haemoblastosis.
The urinary excretion of kynurenine, 3-hydroxykynurenine, kynurenic and xanthurenic acid has been determined by bidimensional paper chromatography in 61 patients with different forms of haemoblastosis (27 cases of Hodgkin's disease, 10 cases of non Hodgkin's lymphomas, 8 cases of acute leukaemia, 11 cases of myeloproliferative disorders, 5 cases of lympho-immunoproliferative disorders). An abnormal urinary excretion of some metabolites of tryptophan's kynurenine pathway is frequent but not constant in all the neoplasias of the myelolymphopoietic system studied so far. In Hodgkin's disease the correlative test between urinary excretion of tryptophan metabolites and clinical stage, histological type and treatment enabled us to point out that the anatomo-clinical diffusion of the lymphoma interferes only through kynurenine and 3-hydroxykynurenine excretion. The histological type seems to influence the 3-hydroxykynurenine excretion. On the contrary, the metabolic alterations present are not affected by treatment. We believe that the metabolic alteration of tryptophan is secondary to a deficit of pyridoxal phosphate and nicotinamide-dependent enzyme activities.
Hematologic Diseases↗
Detection of Epstein-Barr virus genomes in archival tissues by polymerase chain reaction.
We used oligonucleotide primers designed from DNA sequences unique to the long internal direct repeated region of Epstein-Barr virus (EBV) to enzymatically amplify this segment of the EBV genome in formalin-fixed, paraffin-embedded tissues. The products amplified from EBV templates were detected by hybridization with a labeled probe specific for this highly conserved, reiterated region. Epstein-Barr virus-related sequences were detected in the spleen of a patient with infectious mononucleosis, in lung and lymph node specimens from a patient with pulmonary manifestations of infectious mononucleosis, in various tissues from seven immunosuppressed organ transplant recipients with immunoproliferative disorders, and in small biopsy specimens from a patient with nasopharyngeal carcinoma. No viral sequences were detected in 20 histologically normal spleens or 10 lymph nodes. Polymerase chain reaction technology provides an effective means for documenting EBV infection in archival tissues. This approach should facilitate the diagnosis of posttransplant lymphoproliferative disorders and difficult cases of infectious mononucleosis and nasopharyngeal carcinoma.
Adolescent↗
Monoclonal immunoglobulin disorders: a report of 154 cases.
One hundred and fifty-four patients with a monoclonal gammopathy, diagnosed between 1965-1974 in the Hadassah University Hospital are reviewed with special reference to the relative incidence of associated disorders. Most patients (63 per cent) had immunoproliferative disorders (multiple myeoloma, macroglobulinemia of Waldenstrom, chronic lymphocytic leukemia, and other non-Hodgkin lymphomata). A non-B-cell malignancy, either of blood-forming tissues or of epithelial origin, was found in 6.5 per cent. Miscellaneous nonmalignant diseases (chronic liver disease, diseases of known or suspected autoimmune origin, chronic infections, Gaucher's disease), which have been reported in the past in association with a monoclonal gammopathy, were diagnosed in 15 per cent of the patients in this series. Twelve per cent of the patients were either asymptomatic or had diseases not known to be associated with monoclonal gammopathies. Amyloidosis was diagnosed in 3.3 per cent of the patients.
Adult↗
The clinical significance of pure Bence Jones proteinuria at low concentration.
The clinical significance of Bence Jones (BJ) proteinuria at low concentration (less than 0.2 g/24 hours) was investigated in 33 unselected patients who had no intact monoclonal immunoglobulin in their serum. The great majority (79%) of the patients were recognized as having malignant lymphoproliferative diseases, such as chronic lymphocytic leukemia (46%), hairy cell leukemia (6%), and non-Hodgkin's lymphoma (27%), whereas only two patients (6%) had multiple myeloma or systemic amyloidosis. Five patients (15%) had no evidence of definite malignant immunoproliferative disorders at the time of recognition of BJ proteinuria. Three of them, who were excreting steadily low amounts of BJ protein in their urine for 47, 61, and 73 months, respectively, without development of any B-lymphocytic malignancy, were classified as having a monoclonal gammopathy of undetermined significance. In the remaining two patients, BJ protein disappeared spontaneously 14 and 18 months, respectively, after its recognition. The study indicates that pure BJ proteinuria, even when occurring at low concentration, is most consistently associated with malignant proliferations of B-lymphocytic origin. However, the possibility should be considered that the clinical spectrum of the monoclonal gammopathy of the light chain type also includes benign and transient forms.
Adult↗
IgE lambda paraproteinaemia associated with a pleomorphic lymphoproliferative disorder.
A new case of IgE lambda paraproteinaemia is described in which the immunoproliferative disorder was lympho-plasmacytoid, rather than myelomatous in type. The patient, an 81-year-old woman, lacked both organomegaly and bone lesions. The peripheral white blood count was 30 X 10(9)/l (43% lymphocytes and 37% lymphoplasmacytoid cells) and the bone marrow was heavily infiltrated with mononuclear cells with 50% lymphocytes and 50% lymphoplasmacytoid cells). Detailed immunological marker analysis of both peripheral blood and marrow showed an increased population of morphologically normal T cells, together with neoplastic population of B cells. The neoplastic cells were shown to re-express surface immunoglobulin after stripping with anti-E anti-serum, and to synthesis Ig in radio-incorporation experiments. The immunological characteristics of the neoplastic cells are considered in relation to B-cell maturation and it is shown that the immunological phenotype was of the intermediate type to be expected in association with a lympho-plasmacytoid proliferation.
Age Factors↗
Pulmonary cystic disorder related to light chain deposition disease.
Light chain deposition disease (LCDD) is a rare disorder that very uncommonly affects the lung. We report three cases of severe cystic pulmonary LCDD leading to lung transplantation. Such a presentation has never been previously reported. The three patients present with a progressive obstructive pulmonary pattern associated with numerous cysts diffusely distributed in both lungs. The disease was histologically characterized by non-amyloid amorphous deposits in the alveolar walls, the small airways and the vessels. It was associated with emphysematous-like changes and small airway dilation. Monotypic kappa light chain fixation was demonstrated on the abnormal deposits and along the basement membranes. Electron microscopy revealed coarsely granular electron-dense deposits in the same localizations. Mild extrapulmonary deposits were found in salivary glands in one patient. No immunoproliferative disorder was identified. We conclude that LCDD may primarily affect the lung, present as a pulmonary cystic disorder, and lead to severe respiratory insufficiency.
Adult↗
Cryopathic gangrene with an IgM lambda cryoprecipitating cold agglutinin.
Immunochemical and serologic studies of cold agglutinis in patients with chronic cold agglutinin disease (CCAD) have shown the almost exclusive occurrence of IgM kappa antibodies with specificity for the I antigen of red cells. An unusual subgroup of patients has been delineated in which the cryoprotein is IgM lambda, frequently lacks I specificity and often cryoprecipitates. Studies of such a protein from a patient with an unusual array of immunoproliferative disorders including Grave's disease with exophthalmos and Waldenstrom's macroglobulinemia indicate that the cryoprecipitating and cold agglutinating properties probably derive from the sam protein. The occurrence of this type of antibody should suggest the presence of a more aggressive lymphoproliferative disorder than simple CCAD.
Adult↗
Immunoblastic lymphadenopathy and asbestosis.
Immunoblastic lymphadenopathy was diagnosed in a 71-year-old white man who had weakness, with weight loss, fever, cough, and generalized lymphadenopathy. The patient had a long occupational history of shipyard work. Diagnosis of asbestosis was made clinically by chest x-ray, and ferruginous bodies were found in the lung at autopsy. Recent reports have suggested a possible association between asbestos exposure and lymphoproliferative neoplasms. Chronic antigenic stimulation by asbestos could predispose one to the immunoproliferative disorder seen in this patient or be responsible for it. The possible significance of this relationship, previously unreported, is discussed for future consideration.
Aged↗
Association of lymphocytic neoplasia and mesothelioma.
Two lymphocytic neoplasms, chronic lymphocytic leukemia and poorly differentiated lymphoma, were detected in two patients who had nonoccupational exposure to asbestos. Both had a pleural mesothelioma subsequent to their initial presentation with lymphocytic neoplasm. Recent reports have suggested a possible association between asbestos exposure and lymphoproliferative neoplasms. Chronic antigenic stimulation by asbestos could predispose one to the immunoproliferative disorders seen in these patients. The possible significance of this relationship is discussed for future consideration.
Aged↗
Systemic vasculitis in association with human immunodeficiency virus infection.
Fourteen cases of vasculitis associated with human immunodeficiency virus infection have thus far been described. Five of these cases may be classified as angiocentric immunoproliferative disorders, including benign lymphocytic angiitis, lymphomatoid granulomatosis, and angiocentric lymphoma. We report a case of benign lymphocytic angiitis of T cell lineage. Extensive studies found no evidence of viral antigens in the inflammatory infiltrates, and immunologic evaluation of the pathologic lesions revealed the infiltrating cells to be predominantly CD3+, CD8+, CD4-. A significant number of these lymphocytes demonstrated a deletion of T cell antigen receptor determinants. We believe that in certain cases of human immunodeficiency virus disease, there occurs a spectrum of lymphoproliferative disorders with angiocentric features that lead to the clinical picture of systemic necrotizing vasculitis. Clinicians should be aware of this association.
Acquired Immunodeficiency Syndrome↗
Reticulum cell sarcoma of the cecum and macroglobulinemia: a case report.
A case report is presented of an 81-yr old male who developed a reticulum cell sarcoma of the cecum while on immunosuppressive therapy for 13 yr for a primary diagnosis of macroglobulinemia. The considerable overlap between immunoproliferative disorders and the increased incidence of malignancies in patients receiving immunosuppressive therapy are discussed.
Aged↗
Preferential expression of human lambda-light-chain variable-region subgroups in multiple myeloma, AL amyloidosis, and Waldenström's macroglobulinemia.
We have compared the distribution of lambda-light-chain variable-region (V lambda) subgroups among Ig lambda molecules found in the serum of normal individuals with that of monoclonal Ig lambda components obtained from patients with plasma cell and related immunoproliferative disorders. A panel of monoclonal antibodies specific for each of the major human V lambda subgroups--V lambda I, V lambda II, V lambda III, V lambda IV, V lambda VI, and V lambda VIII--was used in a highly sensitive enzyme-linked immunosorbent assay (ELISA) to quantitate each of these populations. The mean distribution of Ig lambda I, Ig lambda II, Ig lambda III, Ig lambda IV, Ig lambda VI, and Ig lambda VIII molecules in serum specimens collected from 20 normal adults was approximately 40, 3, 43, 5, 5, and 3% of the total Ig lambda population, respectively. In contrast, that of monoclonal IgG, IgA, and IgD proteins and Bence Jones proteins obtained from patients with multiple myeloma and related gammopathies (n = 196) was approximately 27, 28, 39, 5, 0, and 1%, respectively. The percentage of monoclonal Ig lambda II components found in individuals with AL lambda amyloidosis (n = 41) was comparably increased to that seen in multiple myeloma and was even higher in patients with Waldenström's macroglobulinemia (n = 16), in whom 63% of the IgM lambda proteins were of the V lambda II subgroup. Also evidenced were differences in the distribution of other V lambda subgroups in the disease states: Most striking was the predominance (41%) of the V lambda VI subgroup among monoclonal lambda chains obtained from patients with AL amyloidosis and that this subgroup was found exclusively on amyloidosis-associated proteins. No Ig lambda VI-type myeloma- or macroglobulinemia-related proteins were identified. The observed alterations in V lambda subgroup distribution among "pathologic" monoclonal Igs were attributed to the particular disease and not related to the heavy-chain class. Our finding that certain V lambda subgroups are nonstochastically expressed in lambda-type multiple myeloma, AL amyloidosis, and Waldenström's macroglobulinemia provides evidence for abnormal VL gene usage in these disorders and, thus, furnishes new insight into their pathogenesis.
Amyloid↗