lymphocyte alveolitis in ham/tsp patients · lymphocyte alveolitis in ham/tsp patients preliminary...

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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 . Dr. Ailto n Mel o — Departament o d e Neuropsiquiatria , Faculdad e d e Medicina , UFB A — Av . Reitor Migue l Calmo n s/ n — 40110-10 0 Salvado r B A — Brasil . Fa x (071 ) 2458562 .

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Page 1: LYMPHOCYTE ALVEOLITIS IN HAM/TSP PATIENTS · LYMPHOCYTE ALVEOLITIS IN HAM/TSP PATIENTS PRELIMINARY REPORT K. MATTOS*, C. QUEIROZ*, A.C.PEÇANHA-MARTINS*, L». PUBL.IO*, V. VINHAS*,

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 .

Dr. Ailto n Mel o — Departament o d e Neuropsiquiatria , Faculdad e d e Medicina , UFB A — Av . Reitor Migue l Calmo n s/ n — 40110-10 0 Salvado r B A — Brasil . Fa x (071 ) 2458562 .

Page 2: LYMPHOCYTE ALVEOLITIS IN HAM/TSP PATIENTS · LYMPHOCYTE ALVEOLITIS IN HAM/TSP PATIENTS PRELIMINARY REPORT K. MATTOS*, C. QUEIROZ*, A.C.PEÇANHA-MARTINS*, L». PUBL.IO*, V. VINHAS*,
Page 3: LYMPHOCYTE ALVEOLITIS IN HAM/TSP PATIENTS · LYMPHOCYTE ALVEOLITIS IN HAM/TSP PATIENTS PRELIMINARY REPORT K. MATTOS*, C. QUEIROZ*, A.C.PEÇANHA-MARTINS*, L». PUBL.IO*, V. VINHAS*,

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 HTLV-I , 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 .

REFERENCES

1. Akizuk i S , Nakazat o O , Higuch i Y , Tanab e K , Setohush i M , Yoshid a S , Miyasak i Y , Yamamoto S , Sudo u S , Sannomiy a K , Okajim a T . Necrops y finding s i n HTLV- I associate d myelopathy. Lance t 1987 , 1:156-157 .

2. Gesaai n A , Vernan t JC , Maur s L , Bari n F , Gou t O , Calende r A , D e Th é G . Antibodie s to huma n T-lymphotropi c viru s type- 1 i n patient s wit h tropica l spasti c paraparesis . Lance t 1985, 2:407-410 .

3. Grimald i LM , Roo s RP , Devar e SG , Case y IM , Maru o Y , Hamad a T , Tashir o K . HTLV- I associated myelopathy : oligoclona l immunoglobuli n G band s contai n anti-HTLV- I p2 1 antibodies. An n Neuro l 1988 . 24:727-731 .

4. Jacobso n S , Gupt a A , Mattso n D , Mingiol i E , McFarli n DE . Immunologica l studie s i n tropical spasti c paraparesis . An n Neuro l 1990 , 27:149-156 .

5. Meirele s A , Moreir a E D Jr , Moreno-Carvalh o OA , Badar ó R , Mel o A . HTLV- I associate d myelopathy i n Salvado r (northeaster n Brazil) . Ar q Neuro-Psiquia t (Sã o Paulo ) 1992 , 50:18^-190.

6. Mel o A , Mour a L , Rio s S , Machad o M , Cost a G . Magneti c radiologi c imagin g i n HTLV- I associated myelopathy . Ar q Neuro-Psiquia t (Sã o Paulo ) 1992 , submitted .

7. Osam e M , McArthu r JC . Neurologi e manifestatio n o f infectio n wit h huma n T cel l lym -photropic viru s typ e I . I n Asbur y AK , McKhan n GM , McDonal d W I (eds) : Disease s of th e nervou s system : clinica l neurobiology . Philadelphia : Saunders , 1992 , 1331-1339 .

8. Roma n GC . Th e neuroepidemiolog y o f tropica l spasti c paraparesis . An n Neuro l 1988 , 2 3 (Suppl) :113-130.

9. Sugimot o M , Nakashim a H , Watanab e S , Uyam a E , Tanak a F , And o M , Kawasak i S . T-lymphocyte alveoliti s i n HTLV- I associate d myelopathy . Lance t 1987 , 2:1220 .