LYMPHOCYTE ALVEOLITI S I N HAM/TS P PATIENT S
PRELIMINARY REPOR T
K. MATTOS* , C . QUEIROZ* , A.C.PEÇANHA-MARTINS* , L» . PUBL.IO* , V . VINHAS* , A . MELO *
SUMMARY — H.TLV I associate d myelopath y ha s bee n describe d a s a systemi c diseas e charac -terized b y manifestation s i n severa l organ s outsid e th e nervou s system . W e repor t inflammator y pulmonary involvemen t i n patient s wit h diagnosi s o f HAM .
KEY WORDS : HT'LV-I , HAM/TSP , alveolitis , pulmonar y involvement .
Alveolite a linfócito s e m paciente s co m HAM/TSP : registr o prelimina r
RESUMO — Mielopati a po r HTL.V I (HAM ) te m sid o descrit a com o doenç a sistémic a caracte -rizada pel o acometiment o d e vário s órgão s alé m d o sistem a nervoso . Nest e registro , estamo s relatando o envolviment o pulmona r e m paciente s co m HAM .
PALAVRAS-CH AVE: HTLV-I , HAM/TSP , alveolite , acometiment o pulmonar .
HTLV-I 'associate d myelopath y (HAM ) ha s bee n describe d a s a neurologi c diseas e characterized b y spasti c paraparesis , sensitiv e syndromes , periphera l neuropathy , constipation , bladder disturbanc e an d impotenc e o r decrease d libid o 5,s. Ther e hav e bee n som e report s o f systemic non-neurologi c manifestation s a s vasculitis , uveitis , Sjogren' s syndrome , arthropathy , cryoglobulinemia, ichthyosis , polymyositi s an d adul t T-cel l leukemia/lymphom a associate d with HA M 7. Pulmonar y infiltrate s i n HA M patient s wer e firs t describe d i n native s o f Mar -tinique 2. I n Japan , bronchoalveola r lavag e studie s showe d fiv e patients i wit h increase d tota l cell count s an d increase d proportio n o f lymphocyte s i n lavag e fluid , a s compare d wit h dat a found i n health y non-smoki n volunteer s 9 . On e o f thes e patients , wit h a n abnorma l ches t X-ray, ha d transbronchia l lun g biops y tha t reveale d lymphocyt e infiltration s i n th e alveola r structures whic h di d no t resembl e leukemi a cells .
In orde r t o clarif y th e clinica l pictur e an d pathogenesi s o f HTLV I associate d myelo -pathy w e hav e studie d th e pulmonar y involvemen t i n eigh t patient s wit h HAM .
PATIENTS AND METHO D — Seve n wome n an d on e man , wit h age s tha t range d fro m 3 9 to 6 7 years , wer e admitte d i n thi s study . Th e patient s presente d th e basi c clinica l an d CS F features o f HA M a s ha s bee n describe d elsewher e 5 . CS F an d seru m sample s wer e collecte d from th e patient s lan d teste d fo r HTLV- I wit h a commerciall y availabl e enzym e immunoassay ; the specimen s wer e repeatedl y reactiv e i n thi s screenin g tes t an d furthe r confirme d b y Wester n blot.
All patient s wer e submitte d t o pulmonar y evaluatio n b y a certifie d pneumologis t (K.M.) . In eac h patien t wa s performe d hemograma , electrocardiogram , ches t X-ray , arteria l gasometri c studies an d spirometr y a t rest . T o accomplis h th e bronchoalveola r lavag e th e bronchoscop e was wedge d int o a dista l bronchu s an d 30 0 m l o f steril e salin e wa s use d t o recove r th e cell s and epithelia l linin g fluid . Th e bronchoalveola r lavag e wa s carrie d ou t i n a singl e laborator y by th e sam e researche r usin g identica l techniques .
•Federal Universit y o f Bahi a (UFRA) , Salvador . Aceite : 25-setembro-1992 .
RESULTS — On e patien t refere d fatigu e an d breathlessnes s wit h exertio n an d thre e ha d occasional cough . Non e o f th e patient s ha d lun g radiographi c findings . On e o f the m presente d mild hypoxemi a wit h n o carbo n dioxid e retention . Al l th e patient s ha d norma ] hemoglobi n and hematocrit . Ther e wa s a norma l vita l capacit y (VC ) i n si x patient s an d th e rati o o f forced expirator y volum e i n on e secon d t o force d vita l capacit y (VEF1/FVC ) wa s als o norma l in si x o f them . On e patien t ha d mil d decreas e i n V C an d on e ha d mil d decreas e i n VEF1 / FVC. Bronchoalveola r lavag e reveale d a n increase d proportio n o f lymphocyte s i n seve n pa -tients (Fig . 1) .
COMMENTS — I t i s believe d tha t HTLV- I play s a n etiologi c rol e i n the . developmen t o f neurologic an d lymphoproliferativ e disorders . Meanwhile , i t ha s bee n demonstrate d tha t HTLV -I infectio n i s relate d wit h disorder s o f skin , vessels , eyes , muscles , articulation s 7 and , a s we hav e shown , pulmonar y involvement . Th e virulenc e o f HTLVI , a CD 4 lymphotropi c retro virus, migh t b e directl y b y infectin g cell s o f systemi c organ s o r throug h a n immunopatholo gical process . Althoug h i t i s no t clea r wha t initiate s th e inflammatio n i n th e HTLV I infec -tion, man y o f th e proces s o f retroviru s infection s ar e understoo d 3,4. Histopathologi c feature s in HTLV- I includ e proliferatio n o f capillarie s an d perivascula r cuffin g wit h lymphocytes/ . These change s determine s i n th e centra l nervou s syste m sever e lesion s mainl y i n th e thoraci c spinal cord , a s i t ha s bee n show n i n necrops y an d magneti c resonanc e imagin g 1,6. Th e slowly progressiv e cours e point s t o a slo w viru s infection . Th e hig h titer s o f antibodie s against HTLV- I i n seru m an d CSF , polyclona l elevation s o f immunoglobulin , th e increase d proportion o f lymphocyte s i n th e bronchoalveola r lavag e flui d an d respons e t o immune suppressive drug s favo r a immun e mechanis m i n th e pathogenesi s o f thi s pleomorphi c syn -drome.
Our result s sho w bronchoalveola r lymphocytosi s i n patient s wit h HAM . Furthe r an d controlled studie s ar e necessar y t o determin e th e rea l frequenc y o f pulmonar y involvemen t i n patients wit h HAM .
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