Cold agglutinin disease (CAD)with autoimmune haemolytic anaemia: a case report of a coronary artery disease patient

Cold agglutinin disease (CAD) with autoimmune haemolytic anemia is characterized by the production of harmful cold autoantibodies associated with increased red cell destruction during exposure to cold. The treatment of CAD is very difficult and a great effort is required to obtain therapeutic success. Cyclophosphamide is a potent immunosuppressive agent which is widely used in all bone marrow transplantation conditioning regimens for patients with acquired severe aplastic anemia. In this report, we describe the case of a coronary artery disease patient with severe CAD, but without lymphoproliferative disease, in which general measures and immunosuppressive therapies were adopted, there by avoiding blood transfusions.

A doença por aglutininas a frio (CAD) cursando com anemia hemolítica auto-imune (AHAI) é decorrente da produção de autoanticorpos que reagem muito bem a baixas temperaturas, dirigidos contra hemácias autólogas. A habilidade desses anticorpos em destruir as hemácias encontra-se diretamente relacionada à sua capacidade em fixar complemento durante a exposição do paciente a baixas temperaturas. A AHAI por anticorpos frios pode ser idiopática - ausência de doença de base - ou secundária, geralmente associada a desordens linfoproliferativas de células B ou determinados processos infecciosos. A hemólise é intravascular, através de aglutininas da classe IgM, com teste direto da antiglobulina humana positivo para complemento. O tratamento da CAD é difícil, exigindo um esforço contínuo, necessário para se obter sucesso terapêutico. A ciclofosfamida é um agente imunossupressor potente, amplamente utilizado em transplantes de medula óssea, particularmente nos portadores de anemia aplástica. Descrevemos o caso de um coronariopata portador de CAD severa, cuja exploração diagnóstica excluiu doença linfoproliferativa. Adotamos medidas gerais de suporte e terapia imunossupressora, coibindo o uso de hemotransfusões.

Effect of periodontal treatment on oral anticoagulation in patients with heart disease.

UNLABELLED: In recent decades, there have been several studies on the correlation between periodontal disease (PD) and cardiovascular disease, but the influence of PD on the effect of oral anticoagulant drugs has not been reported. OBJECTIVE: To assess the influence of PD on oral anticoagulation in patients with heart disease. METHODS: Dental treatment for patients of the Anticoagulation Clinic of the Instituto Nacional de Cardiologia Laranjeiras (INCL), receiving warfarin as a prophylactic treatment for thromboembolic events, was performed without suspending the drug and according to the INCL's "Protocol of dental treatment for patients with acquired coagulopathy". A therapeutic anticoagulation level was maintained and was assessed using the international normalized ratio (INR) on the of the patient's visit. The patient was thus protected against thromboembolic events and could undergo dental treatment, even oral surgery. Our study comprised 40 patients who underwent prospective oral assessment and were divided into two groups: Group I--20 patients with PD; and Group II--20 patients without PD. Dental treatment was performed in the two groups as follows: PD control in Group I and treatment of dental caries in Group II. The INR of the patients was assessed before each dental consultation, to guarantee hemostasis during the procedures and to monitor the anticoagulation level obtained. INR prior to the dental intervention was then compared with that after the intervention in both groups. An INR increase of > or =50% was considered significant. RESULTS: In Group I, all patients showed an increase in INR after the dental treatment, which was significant in 15 (75%). In Group II, only 8 patients had increased INR, which was significant in 5 (25%) (p = 0.002). Considering the oral health of the two groups,. the extent of tissue injury in the oral cavity was not significant compared to the INR increase; however, comparison between the two groups showed significant INR increase mainly in patients with PD (p = 0.002). CONCLUSION: This study showed that dental treatment in patients with any type of PD significantly increases INR.

Genetic markers on the HTLV-1 p12I protein sequences from Brazilian HAM/TSP patients and asymptomatic HTLV-1 carrier isolates.

The human T cell leukemia/lymphotropic virus type-1 (HTLV-1) genome has approximately 9 kb and contains the pX region that codes for regulatory and accessory proteins. The pX ORF-I encodes for the p12 protein, a 99 aa peptide, which presents several functional putative domains, such as leucine zipper motifs, SH3- binding motifs, and a dileucine motif, p12I. Also, a rare p12IK88 allele was found mainly in HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) patients, suggesting it is a marker of pathogenesis, although recent studies showed p12IK in asymptomatic HTLV-1 carriers. To extend the observations on p12I motifs, we sequenced 26 p12I from HAM/TSP patients and asymptomatic HTLV-1 carriers. Amino acid analysis of 48 p12I motifs demonstrated the presence of several alleles, but the allelic variation, including p12IK, was not prevalent in HAM/TSP isolates. Nonetheless, some genetic markers were recognized in association with isolates from HTLV-1a subgroup B and Brazilian HTLV-1aA strains.

HTLV-II infection associated with a chronic neurodegenerative disease: clinical and molecular analysis.

HTLV II is a retrovirus endemic in some Amerindian tribes and spread worldwide with a high prevalence among intravenous drug abusers. It has three different genetic subtypes a, b, and d, defined mainly by the long terminal repeat (LTR) region. HTLV II has been associated with a neurodegenerative disease in few cases. We describe the first well-documented case in Brazil where the virus is endemic in isolated ethnic groups. The patient is a 55-year-old woman with a chronic and painful syndrome characterized by spastic paraparesis, hyperactive reflexes and spastic bladder. Somatosensory evoked potential indicates a thoracic spinal cord lesion. Computer tomography showed periventricular demyelination. Enzyme-linked immunosorbent assay was positive for HTLV I/II whereas the discriminatory Western blot was indeterminate. Molecular analysis of the Tax region revealed a HTLV II pattern that was also confirmed through sequencing the LTR region. Phylogenetic analysis of the LTR sequence shows an HTLV IIa subtype that clustered with the virus isolated from Kayapo Indians and Brazilian urban intravenous drug users. Indeterminate Western blots are frequently found using commercial kits, therefore we recommend that all cases in which a myelopathy is associated with an indeterminate serological result should be evaluated by PCR to determine the actual number of HTLV II associated myelopathy cases.