Immunomodulatory treatment with low-dose interferon-alpha and oral retinoic acid in lymphangioma-like Kaposi's sarcoma.

BACKGROUND: The presence of lymphangiectasis without the characteristic spindle cell proliferation may lead to diagnostic difficulties in Kaposi's sarcoma. Although the literary data mention that the lymphangioma-like tumors may occur in Kaposi's sarcoma, there have been few specific reports and case presentations published. OBSERVATIONS: A case of lymphangioma-like Kaposi's sarcoma in association with IgG/lambda type paraproteinaemia is reported in a 60-year-old man. The HSV8 DNA sequence could be detected by PCR analysis from lesional skin. CONCLUSION: The beneficial effect of alpha-2 interferon (4.5 million units per week) combined with retinoic treatment (0.5 mg/body weight of isotretinoin) caused the regression of the skin rashes while improving the values of immunological tests (T cell function, quantity of paraproteins). The patient's improved general condition and the ameliorating immunological parameters were due to the combination of two regimens applied in a low-dose the alpha-2 interferon (tumor regression) and the oral isotretinoid (antitumor activity, reduction of IL-6 receptor display) treatment.

Trasplante de médula ósea en pacientes portadores de inmunodeficiencias severas combinadas / Bone marrow transplantation in patients with severe combined immunodeficiency

La inmunodeficiencia severa combinada (IDSC) es una inmunodeficiencia primaria cuya evolución clínica es mortal si no se realiza un tratamiento con trasplante de médula ósea (TMO). En los últimos 8 años cuatro pacientes de sexo masculino han sido sometidos a TMO, dos de ellos con donante idéntico (hermanos) y los otros dos con médula obtenida de sus respectivas madres (haploidéntico). Como elementos de importancia deben mencionarse la presencia de diseminación de la vacuna BCG en dos de ellos, una paraproteinemia de predominio IgM en un caso y la identificación de una quimera por linfocitos maternos en otro niño. En los cuatro casos existía compromiso pulmonar de importancia, con secuelas tipo bronquiectasias en dos de ellos. Dos pacientes que recibieron TMO idéntico normalizaron su trastorno inmunológico al mes y tres meses de haber recibido el TMO, encontrándose en la actualidad sanos con un tiempo de seguimiento de 1 y 7 años respectivamente. Los otros dos enfermos trasplantados con médula no idéntica fallecieron de complicaciones atribuibles a infecciones y al daño pulmonar existente, al 1 y 2 meses de efectuarse el TMO. Las implicancias prácticas del diagnóstico de la IDSC y los aspectos más importantes del TMO son comentados

[Monoclonal gammopathy and primary hyperparathyroidism. Apropos of 2 cases and review of the literature]. / Gammapathie monoclonale et hyperparathyroïdie primitive. A propos de deux observations et revue de la littérature.

Frequency of primary hyperparathyroidism (HP) is increased in neoplasia. Though uncommon, both HP and monoclonal gammopathy are not exceptionally associated as seen in a literature review reporting 24 observations. The mechanism of this association remains unclear but does not seems coincidental. Association of hypercalcemia with gammopathy does not suggest systematically malignant hypercalcemia and implies the search for hyperparathyroidism. The authors report two patients presenting respectively a multiple IgD myeloma and an monoclonal gammopathy of undetermined signification associated with a parathyroid adenoma.

Monoclonal gammopathy associated with Crohn's disease during treatment with total parenteral nutrition.

A patient with Crohn's disease developed monoclonal gammopathy while receiving total parenteral nutrition. There was no evidence of worsening of the primary disorder or presence of infection or malignancy.