1/5. Cold agglutinin disease--the importance of cutaneous signs.We present the case of a woman where the diagnosis of cold agglutinin disease could be made after we had noticed slight cutaneous manifestations during a routine examination. Leading symptoms were livedo reticularis of the thighs and a history of acrocyanosis and Raynaud's phenomenon upon cold exposure. The current knowledge about the etiology, clinical presentation and treatment of the disease is briefly discussed.- - - - - - - - - - ranking = 1keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
2/5. Cold haemagglutinin disease complicating mycoplasma pneumoniae infection in a child under cytotoxic cancer treatment.Acute cold haemagglutinin disease, most commonly associated with underlying mycoplasma infection, is rare in children. A 3-year-old girl who developed this auto-immune disease under intensive cytotoxic treatment for rhabdomyosarcoma is presented. Clinically, a livedo reticularis skin pattern upon exposure to cold which was reversible at room temperature and a spontaneous red cell agglutination of blood samples in vitro led to the diagnosis. Together with bronchopneumonia the girl developed hyper-IgM, high antibody titres against mycoplasma pneumoniae, as well as high titres of cold agglutinins. Laboratory signs of mild intravascular haemolysis were found. Positive direct antiglobulin test resulted from coating of red cells with C3d and C4. Three different antibodies were identified in serum: nonspecific cold agglutinins without complement activation, anti-I specific cold agglutinins with complement activation, as well as a weak biphasic Donath-Landsteiner haemolysin. Under antibiotic treatment and a short course of predisolone the clinical course was mild.- - - - - - - - - - ranking = 1keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
3/5. Anti-Pr cold hemagglutination associated with livedo reticularis.A report of anti-Pr cold hemagglutination associated with livedo reticularis is presented. livedo reticularis is descriptive of the superficial venous system appearing when blood flow is altered in this area. Cold antibody directed against the Pr antigen of the red cell membrane can cause hemagglutination, thereby altering blood flow in cooler areas of the body. This patient had livedo reticularis approximately 8 months prior to detection of the anti-Pr cold agglutinin.- - - - - - - - - - ranking = 6.2102391907732keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
4/5. Anti blood group-M autoantibodies with livedo reticularis, Raynaud's phenomenon, and anaemia.A 63-year-old woman presented with Raynaud's phenomenon and extensive cold-induced livedo reticularis. A skin biopsy showed no abnormality of the blood vessels but the blood contained high titres of a very unusual autoantibody against the M blood group, most active at low temperatures. An IgM cryoglobulin was detected, and anti-M activity was found in this fraction. The cells of the patient were grouped as MM. The direct antiglobulin test was positive due to C3 component of complement bound to the red cells. The haematological and biochemical results indicate a mild haemolytic process, which is at present well compensated.- - - - - - - - - - ranking = 5keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
5/5. Anti-Sa cold agglutinin of IgA class requiring plasma-exchange therapy as early manifestation of multiple myeloma.The case of a patient (Col) with multiple myeloma presenting as chronic cold agglutinin (CA) syndrome is reported. The CA (Col) was a monoclonal IgA/k paraprotein which recognizes an antigen fully expressed in adult and newborn erythrocytes, sialidase sensitive and partially resistant to proteases. hemagglutination-inhibition studies showed that immunodominant n-acetylneuraminic acid bound alpha 2-->3 to O-glycans of glycophorins represents the CA(Col) epitope. These serological and biochemical findings fit with the anti-Sa specificity, of which only two previous examples are known. The clinical manifestations of CA (Col) were characterized by marked acrocyanosis, generalized livedo reticularis, and incapacitating dyspnea, but only mild hemolysis. Plasma-exchange therapy was effective in quickly removing the CA and relieving the associated clinical manifestations, but such benefit was only temporary. This is the first reported example of anti-Sa CA of IgA isotype and the first case of IgA CA syndrome treated by plasma exchange.- - - - - - - - - - ranking = 1keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |