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.
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.
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. Rev. bras. hematol.
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