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Biomedical subjects

F Danon

Publications and source records attributed to F Danon.

At least 91 records · Page 5Linked to original sources
PubMed1978

Gamma heavy chain disease simulating alpha chain disease.

A young Turkish girl presented with all the clinicopathological features of a digestive form of alpha chain disease. A gamma heavy chain disease protein, however, was found in her serum and also in the cells invading the intestinal mucosa and mesenteric lymph nodes.

Child↗
PubMed1977

[Hereditary C2 deficiency with systemic lupus erythematosus: clinical and immunologic studies in a family (author's transl)].

A case of systemic lupus erythematosus associated with an homozygous deficiency in the second fraction of complement is reported and compared to previous reports of the literature. The high incidence of infections in these patients is outlined. The defective gene in this family was associated with the HLA A10B18 haplotype and the propositus was homozygous at the HLA-D locus. Familial study allowed the detection of 3 heterozygous individuals two of them being symptomatic (vascular purpura, high incidence of bacterial infections).

Adolescent↗
PubMed1977

Clinical and pathologic features of Waldenström's macroglobulinemia in seven patients with serum monoclonal IgG or IgA.

The clinical, hematologic and pathologic findings in seven patients were similar to those of Waldenström's macroglobulinemia, but unexpectedly the serum monoclonal immunoglobulin belonged to the IgG class in five patients and to the IgA class in two. The bone marrow and lymph node lymphoid proliferation was pleomorphic, with the simultaneous presence of small lymphocytes, normal mature plasma cells and transitional lymphoplasmacytic cells. Immunofluorescence studies showed that a monoclonal immunoglobulin similar to that found in the serum was detectable on the membrane or in the cytoplasm of all the proliferating cells, which thus belonged to the same B cell clone. The study of these patients is in accordance with the concept that lymphoid disorders featured by a pleomorphic monoclonal B cell proliferation constitute a distinct clinicopathologic entity, which is not restricted to IgM-producing clones.

Adult↗
PubMed1976

Intracytoplasmic and surface-bound immunoglobulins in "nonsecretory" and Bence-Jones myeloma.

Immunoglobulins were studied at the cellular level by direct immunofluorescence in twenty-five patients with 'nonsecretory' myeloma and thirty-six patiens with Bence-Jones (BJ) myeloma. The results were compared with those obtained in a control group of thirty-six patients with common secretory myeloma. A monoclonal Ig (IgG in eighteen, IgA in three and kappa chains only in three cases) was found in the cytoplasm of the plasma cells from all the patients with 'nonsecretory' myeloma, with a striking dysbalance in the staining brightness for the heavy and the light chains. A similar dysbalance in staining was also observed for plasma cell surface Ig chains but in the opposite way. In twenty patients with BJ myeloma studied for cytoplasmic Ig only, determinants of a heavy chain were clearly found in four cases. When surface Ig were studied also, the production of gamma chains in addition to the light chain could be ascertained in six of sixteen cases. In addition, IgM with the same light chain type as the BJ protein was detected at the cell surface on plasma cells and lymphocytes in two of these sixteen patients. 'Monoclonal' populations of B lymphocytes bearing the same Ig chains as those produced by the myeloma cells were detectable in five of eleven 'nonsecretory' myeloma and in five of sixteen BJ myeloma patients. Normal blood B lymphocytes were in decreased number, particularly when a 'monoclonal' lymphocytic population was detected. Data are discussed which suggest that plasma cells from most patients with 'nonsecretory' myeloma might synthesize and secrete Ig molecules with structurally abnormal chains that are then quickly degraded.

Bence Jones Protein↗
PubMed1975

A new case of heavy mu-chain disease.

The tenth case of mu chain disease is described. The patient lives in Ivory Coast as in our previously reported case. He was not affected with chronic lymphocytic leukaemia and the main clinical feature was liver cirrhosis of unknown origin. The amount of abnormal protein in the serum was great enough to give an abnormal bond on the routine electrophoresis. The protein was devoid of light chains and was present in the form of disulfide linked polymers of incomplete mu chain. The molecular weight of the monomer was approximately 58,000. The protein comprised the Fc fragment and a part of the Fd segment. Bence Jones protein was not found in the urine.

Cote d'Ivoire↗