Guía de laboratorio para el diagnóstico y seguimiento de pacientes con gammapatías monoclonales / Laboratory guidelines for the diagnosis and follow-up of patients with monoclonal gammopathies

Se presenta una guía elaborada por el grupo de Inmunoquímica de la Sociedad Española de Inmunología con el objetivo de proporcionar una herramienta práctica para el diagnóstico y seguimiento de las gammapatías monoclonales. Se revisan las características clínicas y analíticas de los diferentes tipos de gammapatía monoclonal, las guías de consenso internacionales y las técnicas utilizadas para la detección y seguimiento del componente monoclonal (AU)

We present guidelines from the Immunochemistry group of the Spanish Society for Immunology that are designed to provide a practical tool for the diagnosis and follow-up of monoclonal gammopathies. We review the clinical and analytical features of various monoclonal gammopathies, international consensus guidelines and techniques used to detect and follow-up monoclonal components (AU)

Primary plasmacytoma involving mediastinal lymph nodes: A diagnostic mimicry of primary mediastinal lymphoma.

Plasmacytomas could involve any organ, and at times might pose a diagnostic challenge when the site of involvement is unusual, or if the presentation is similar to other diseases. We describe a 48-year-old man presenting with worsening shortness of breath and chest discomfort with radiologic evidence of mediastinal enlargement, mimicking a lymphoma with mediastinal involvement. An excisional biopsy of a mediastinal lymph node showed a plasma-cell infiltrate strongly positive for CD138, with a flow-cytometry analysis showing a population of lambda-restricted neoplastic plasma cells. He failed to respond to 50Gy involved-field radiotherapy, but achieved a partial response to combination chemotherapy. He underwent high-dose chemotherapy with melphalan (200mg/m(2)) followed by lenalidomide maintenance, and is in complete remission 18months postautografting. This case illustrates a unique and rare presentation of primary lymph-node plasmacytomas involving the mediastinum potentially mistaken as lymphoid malignancy. Clinicians should be aware of the plasma-cell origin of the mediastinal neoplastic process.

Epidemiology of the plasma-cell disorders.

This review of the plasma-cell disorders begins with the definition of monoclonal gammopathy of undetermined significance (MGUS). The prevalence of MGUS in white and black populations is described. MGUS is a common finding in the medical practice of all physicians, and thus it is important to both the patient and the physician to determine whether the monoclonal protein remains stable or progresses to multiple myeloma (MM), Waldenström's macroglobulinemia (WM), primary systemic amyloidosis (AL), or a related disorder. The long-term (almost 40 years) follow-up data of 241 patients in the Mayo Clinic population is provided. In a large study of 1384 patients with MGUS from southeastern Minnesota, the risk of progression to MM, WM, AL, or other disorders was approximately 1% per year. Risk factors for progression are provided. The incidence of MM in Olmsted County, Minnesota, remained stable for the 56-year span 1945-2001. The apparent increase in incidence and mortality rates among patients with MM in many studies is due to improved case ascertainment, especially among the elderly. The incidence and mortality rates of MM in the United States and other countries are presented. The major emphasis is on the cause of MM, which is unclear. Exposure to radiation from atomic bombs, therapeutic and diagnostic radiation, and in workers in the nuclear industry field are addressed. Many studies involving agricultural occupations, exposure to benzene, petroleum products, and engine exhaust and other industrial exposures are discussed. Tobacco use, obesity, diet, and alcohol ingestion are all possible causes of MM. Clusters of MM have been noted. Multiple cases of MM have been found in first-degree relatives.

Hepatic and renal plasma cell lesions in a patient with multiple myeloma in hematological remission.

Plasma cell myeloma is characterized by plasma cell infiltrations, the presence of paraprotein and mostly skeletal destruction with osteolytic lesions. Liver or kidney infiltration has been described in patients with end stage multiple myeloma (MM), while the features of this malignant dissemination is not clearly understood. An atypical form of MM relapse is presented in a patient being in bone marrow remission, with simultaneous lesions in liver, kidney and suspicion of brain involvement. This case is an interesting model of refractory MM extraosseous involvement, that a clinician should be aware of when carrying out differential diagnosis.

Plasma cell infiltration of the upper aerodigestive tract treated with radiation therapy.

A case of chronic, fluctuating plasma cell gingivostomatitis that progressed despite chemotherapy and surgery is reported. This is the first case reported of treatment with radiation therapy, and one of the few cases reported where the infiltrate has reached the larynx. After receiving low dose radiation therapy, via a conformal technique encompassing the respiratory mucosal lining from the base of tongue to carina, there has been symptomatic improvement.

[Lymphoid tissue of mice spleen in the delayed periods following a single gamma-irradiation]. / Limfoidnaia tkan' selezenki myshei v otdalennye sroki posle odnokratnogo gamma-oblucheniia.

Morphofunctional state and recombination of cell composition of the spleen structural components were studied in mice Balb/c at delayed (post-irradiation days 21, 30, and 58) periods following a single gamma-irradiation by 6.9 Gy. At none of these time points did the lymphoid structural components of the spleen complete their morphological and cytological recovery. Morphological disturbances included poor development of germinative centers and marginal zones in lymphadens and periarterial lymphoid heads (PALH). Recovery of lymphoid cell population did not go on same pattern. Thus, by day 58 of recovery, in PALH small lymphocytes regained basal level but rose concentration to 79% in the lymphadens without germinative centers and to 60% in the lymphoden mantle and red pulp. Elevation of mitotic and cell differentiation activities was not equal. Destructive processes approached normal values in PALH and red pulp and remained by 85% beyond basal values in lymphodens. Mature/immature plasmatic cells ratio was modified. Moreover, plasmocytes (mature cells) that are responsible for humoral immunity, localized in unordinary sites, i.e. in PALH and lymphodens without germinative centers. Adaptive changes observed in the red pulp at delayed time-points after irradiation were manifested by development of myelopoiesis focuses with high concentrations of young forms of neutrophils and megakaryocytes.

Ex vivo optical properties of human colon tissue.

Using a spectrophotometer equipped with an internal integrating sphere, the absorption (mu a) and the reduced scattering (microseconds') coefficients of ex vivo human colon tissues were evaluated from reflectance and transmittance measurements. Mu a and microseconds' varied from 47.7 to 1.0 cm-1 and from 14.2 to 6.2 cm-1, respectively, on passing from 300 nm to 800 nm. These results can be used to estimate the optical penetration depths when photodynamic therapy or light-induced fluorescence procedures are used.

[Morphometric cellular characteristics of the inflammatory infiltrate in nonirradiated and irradiated rectal cancer]. / Morfometricheskaia kharakteristika kletok vospalitel'nogo infil'trata neobluchennogo i obluchennogo raka priamoi kishki.

A morphometric analysis of the inflammatory infiltrate cells of the stroma and adjacent tissue of adenocarcinomas of the rectum was performed in 40 patients. As to therapeutic modality, the patients were divided into 4 groups of 10 patients in each. The 1st group was given surgery only, the 2nd group preoperative gamma-therapy at a dose of 19.5 Gy, the 3rd 30 Gy and the 4th 40 Gy. Integral fractions of lymphocytes, plasmocytes and eosinophils were determined on randomly selected sections using the point counting technique. It was shown that in adenocarcinomas of the rectum integral fractions of the above cells had similar values irrespective of tumor macroscopic shape, degree of differentiation and growth depth. Cellular reactions in the adjacent tissues were more noticeable than in tumor itself, and numerical values of the integral fractions of inflammatory infiltrate cells depended on growth depth. After irradiation a decrease in the lymphocyte integral fraction and an increase in the plasmocyte integral fraction were noted both in the stroma and adjacent tissues.

Studies of long-term continuous irradiations using daily doses ranging from 0.07 to 0.30 Gy on the B lymphoid system of the rat.

The effects of a continuous exposure to cobalt gamma rays administered to rats at a daily dose of 0, 0.07, 0.12, 0.20 or 0.30 Gy for a period of up to 90 or 135 days, have been observed on their B lymphocyte populations and on their immunoglobulin serum levels. The effects increase with the daily dose and the duration of irradiation. At a daily dose of 0.07 Gy, no clear effect was observed. The depletion was almost negligible after 30 days at a daily dose of 0.12 Gy, but visible after all other doses and durations. However, a clear difference in susceptibility was observed between the marginal zone B compartment and the follicular one, the former being much more affected by the radiation than the second.

[Ultrastructure of the lymph nodes of dogs under prolonged external gamma irradiation]. / Ul'trastruktura limfaticheskikh uzlov u sobak pri dlitel'nom vneshnem gamma-obluchenii.

The purpose of the present investigation was electron microscopy study of lymph nodes of dogs exposed to chronic gamma-irradiation during 6 years (with a total dose of 125 rad/year). The exposure induced changes in the cell composition of the paracortical regions due to a decrease of the count of small lymphocytes and a predominant increase of young blast cells with an altered ultrastructure. Chronic gamma-irradiation led to an increase of the number of plasma cells and emergence of intermediate cell forms due to "plasmatization" of lymphocytes and reticular cells.

The effects of continous irradiation on histological picture and deoxyribonucleoprotein content in spleen of rats.

The histological picture and deoxyribonucleoprotein (DNP) content in the spleen of the white male Wistar rats continuously irradiated with the daily dose rates 9.57 mGy (1 R), 95.7 mGy (10 R), 478.5 mGy (50 R) and 657.0 mGy (100 R) were studied. The animals were examined within the day until 60 of irradiation. The number of lymphocytes permanently decreased at the daily dose rates 95.7 mGy and higher ones. The increased activity of reticulum with the simultaneous multiplication of the erythroid cells in the red pulp of spleen was observed at day 10 and 60 of irradiation with 9.57 mGy and 95,7 mGy. Simultaneously DNP content in spleen increased in accordance with the increased activity of reticulum and extramedullar erythropoiesis. The significant decrease in DNP content alter the onset of irradiation with the daily dose rates 478.5 mGy and 957.0 mGy was caused mainly by the fall of lymphocytes. The incidence of eosinophilic leukocytes increased from day 40 irradiation with the daily dose rates 9.57 mGy, 95.7 mGy and 478.5 mGy and decreased with the daily dose rate 957.0 mGy. The plasma cells tended to increase in accordance with the increasing accumulated dose of irradiation with all daily dose rates.

Microwaves induce an increase in the frequency of complement receptor-bearing lymphoid spleen cells in mice.

A single 30-min exposure of mice to 2450 MHz microwaves (12 to 15 mW/g body weight) in an environmentally controlled waveguide facility induced a significant increase in the proportion of complement-receptor positive lymphoid cells in the spleen. This effect was further enhanced by repeated (three times) exposures, which in addition produced a significant increase in the proportion of Ig+ cells. The proportion of theta-positive cells and the total number of spleen cells remained unchanged.

[Alterative and repair processes in lymph nodes under the direct and mediated effects of radiation]. / Al'terativnye i vosstanovitel'nye protsessy v limfaticheskikh uzlakh pri priamom i oposredovannom deistvii radiatsii

Under study were the popliteal lymph nodes of rats at different times after total irradiation of animals (the 1st series), total irradiation with screening the left node (the 2nd series) and local screening of other parts of the body (the 3rd series). X-ray irradiation in all experiments was performed under standard conditions in dosage of 800 r. The amount of mitoses (MC 0/00) in light centers and cortical substance was counted in addition to histological alterations. In shielded lymph nodes (2nd series) mediate effects of irradiation were observed characterized by a decrease of the MK amount and massive death of lymphocytes in later terms than after direct effects (1st series). In irradiated nodes (3rd series) the reparative process was more rapid than in the first series due to migration of lymphocytes from non-irradiated parts of the body. The mediate effect of radiation results also in increased amount of plasma cells in lymphatic nodes of animals subjected to total irradiation (1st and 2nd series). It is suggested by the absence of such increase of amount of plasma cells in locally irradiated lymphatic nodes when screening other parts of the body (3rd series). availability of individual distinctions in the character of the lymphoid tissue response to effects of ionizing radiation puts a question of division of experimental animals at least into 2 subgroups which have different indices of proliferative processes.