Journal of the Royal Society of Medicine Volume 85 July 1992 6 Kim P, Ebersold MJ, Onofrio BM, Quast LM. Surgery of spinal nerve schwannoma. Risk of neurologibal deficit after resection of involved root. J Neurosurg 1989,71810-14. 7 Zbieranowski I, Bedard YC. Fine needle aspiration of schwannomas. Value of electron m Py and immunocytochemintry in the preoperative diagnoi Ata Cytol 1989,3:3814 8 Dayan D, Buchner A, Hirschberg A. Ancient neurilemmoma (Schwannoma) of the.oral cavity. J Craiomaxillofac Surg 1989;17:280-2 9 Gray MH, Smoller BR, McNutt NS, HuA. A mUnoi chemical demonstration- of factor XIIla expressis in neurofibromas. A practical means of difertiating thebe tumour8
417
from neuroticed melanocytic nevi and schwanxomas. Arch
Dermaftl 1990;126:472-f6 10. Davidso SF, Das SK Srith,EE. Cellular schwannoma of the hand J4 Hand Surg [Am] 1988;14:907-9 .11 RabsdgSA Browse NL, Tighe JR, Fklher CD. Malignant nerve sheath tumour arising in a benign ancient schwannoma. Hietopathology 1989;14:525-8 12 Benzel EC, Morris DM, Fowler MR. Nerve sheath tumors of the sciatic nerve and sacral plexus. J Surg Oncol 1988;39:8-16
(Accepted 28 January 1992)
Chronic adult T-celIlkm ymphoma presenting with cutaneous mifestations
Case presented to Section of
Dermatology, 20 June 1991 24Kb w6L rw%nn 14Kb_ 9.5Kb_
W G Phillips MRCGP MRCP1 J A A Langtry BSc MRCP1 C Formstone BSc PhD2 A M R Taylor BSc PhD2 J R Marsden BSc MRCP1 1Department of Dermatology, The General Hospital, Steelhouse Lane, Birmingham B4 6NH and 2Department of Cancer Studies, Birmingham University, Birmingham B15 2TJ
'lif1rb,~)94I;
Keywords: adult T-cell leukaemia/lIymphoma; HTLV-1; poikiloderma
Mtow>Xelt.9K
b
2
3
I. Barn Hl beta chain ,Y' T5&9%Woior C C beta chain ;rn{;
Figwre2. (i-This Southern blot ofaBamHl digest ofDNA was probed ators for the T-ceLl receor C beta chain gene Lane us the DNMAshows the.germline band at 24 kg. Lane 2.shows DNA from
lymphocytosis.
a,T-cel
clone cutured in vitro. The germline band is lost and two
rearranged kqnde representing the two e iag lles of the
Case report A 67-year-old Afro-Carribean woman presented with an 8 month history of ger,eraized pruritus ndmalaise. She had been resident in the United Kingdom for 30 years and had no previous illness of note. There was no, history otblood transfusion, intravenous drug abuse or sexual activity with a high risk for acquiring WV., On examination she had widespread cutaneougs atrophy, telangiectasia, and reticulate hyperpigmentation characteristic of poikiloderma and widespread monomorphic dermal papules most marked on the face. There was 2-3 cm axillary, inguinal, and supraclavicular lymphadenopathy. There were no other abnormal physical findings, in particular there was
T-eUl C beta chain gene are present. Lane 3 shows DNA from the pdtients ymphoytes which show the germline band at24 kb and two rearranged bands at 14 kb and 26 kb showing thepresence ofa single T cel clone in peripheral blood. ( This Southern blot of an EcoRl digest of DNA- has been probed for HTLV-I. Lane 1 shows the inestigator DNA; The band at 8 kb represents a normal human sequenc and iua recognized artifact generated at the time of cloning the ITL V-i priobe. Lane 2 8hows DNA from the lymphoes of a paticet with GAuteATLL while laneS3shows DNA from our patients lymphocytes An extra band an b-seen in bbth hes2 and proing the presence of monoclonally integrated HTLV-I proviral DNA in both patients
no hepatomegaly or splenomegaly and there were no upper motor neurone signs in the lower limbs. Investigation revealed a lymphocytosis of 13x 1O9/1, 70% of which were CD3-ve CD4+ve s*Ua lymphqcytes. Skin -bopsy showed, an inffltra ofcytolpgically normal sa lnpho.ytea in te papi4lary derm wjrg h ei otropimd 'i. telkLymph nodeitgy h nsionoozmi4tent with T cell lymphomA. nne marrov , cpa
_
Z ..
'45
.
-
A
^. p
_
J
Figure 1. Biopry of poilod*ematous abdbmina shin *howingun infiltrate of cytologwally normal msnal lymphocytes gn he papikiry dermis with epiderotropism (H & E, xl1O00)
-
aspiate cytology showed- lymphoid infiltate comprising 40* of nuleated cello.,Soia of these cells had clQvOn nuaclei and 10% were immature. Serum cal a te dehydrgenase, skeletal a. and lymph te bromosopies were normal. Anti _HTLV-1 antibody iFas positive _y ELISA. T-cell receptor gene rearrangement s es ir dan oa al ToeU clonel is blood (ige 2). 4rder A show thatITLV l and te prec -of is related- to our patient's di anti-dHTLV-1 antibody was not an incidental finding we demoPtathe integration of HTLV-1 proviral DNA into the tumwourgenome. Ti was done by, Sothrn blotting an ECoR1 digest of DNA extracted from peripheral blood
Q14X-0768/92/ 070417rO2/$02.000/ i 1992 The Royal Society of Medicine
418
Journal of the Royal Society of Medicine Volume 85 July 1992
lymphocytes and probing for HTLV-1 (Figure 2). As there are no EcoRl restriction sites within the provirus1 and the provirus inserts at random within the genome of the hosts T-cells2 the presence of an extra band on this study shows that HTLV-1 proviral DNA is incorporated within the genome of a T-cell clone. Discussion
Monoclonal integration of the HTLV-1 proviral genome is associated with a spectrum of lymphoproliferative disease. Pre-ATLL is an asymptomatic lymphocytosis of < 10 000 without clinical evidence of organ infiltration3. Smouldering ATLL involves cutaneous infiltration without lymphocytosis. Chronic ATLL consists of lymphocytosis and cutaneous involvement without visceral disease. Acute ATLL is characterized by large numbers of leukaemic cells in peripheral blood, cutaneous, lymph node, bone and visceral involvement with poor prognosis4. Although cutaneous infiltration is a well recognized presentation of ATLL4-7 this is the first patient we are aware of with chronic ATLL who has presented with the combination of poikiloderma and leukaemic cells with the cytological appearance of normal small lymphocytes in peripheral blood.
Ulcerative colitis and bleeding from a colonic vaginoplasty
T W Hennigan FRCS N A Theodorou FRCS Department of Gastrointestinal Surgery, Charing Cross Hospital, Fulham Palace Road, London W6 8RF Keywords: ulcerative colitis; vaginoplasty; gender reassignment
Vaginal reconstruction using a segment of sigmoid colon is not a new procedure and is occasionally performed after gender reassignment surgery or following vaginal excision for carcinoma of the vagina. A case is presented in which such a patient developed ulcerative colitis in the colon and rectum and also developed similar inflammatory changes in the isolated section ofcolon in the vaginoplasty.
Case report A 26-year-old man had a gender reassigmnent 16 years ago having lived in a female role for 18 months. The patient developed a 'vaginal' prolapse which-was repaired twice but recurred. A vaginoplasty using approximately 12 cm of sigmoid colon was performed in 1980. In 1990, the patient presented with a 2 month history of rectal bleeding and diarrhoea with 10-15 bowel actions per day associated with colicky abdominal pain and weight loss of half a stone. In addition, a blood-stained 'vaginal' discharge had been noted. On examination the patient was pale with a tachycardia of 130 beatslminute but no pyrexia. The abdomen was distended with tenderness in the left iliac fossa. Sigmoidoscopy demonstrated contact bleeding and extensive ulceration. Examination of the neovagina at that Correspondence to: Mr T W HeDnigan, 125 Balfour Road, London W13 9TW
References 1 Yoshida M, Miyoshu I, Hinuma Y. Isolation and charcterisation of retrovirus from cell lines of human adult T-cell leukemia and its impcan in the diease. Proc Nati Acad Si USA 1989,7.2031-5 2 Seiki M, Eddy R, Shows TB, Yoshida M. Nonspecific integration ofthe HTLV1 provirus genome into Adult T-cell leukaemia cells. Nature 1984;309:640-2 3 Kinoshita K, Amagaski T, Ikeda, et aL Preleukemic state of adult T-cell leukemia: abnormal T lymphocytosis induced by human adult T-cell leukemia/lymphoma virus. Blood 1985;66:120-7 4 Kawano F, Yamaguchi K, Nishimura H, Tsuda H, Takatsuki K. Variation in the clinical course of Adult T-cell leukemia. Cancer 1985;66:851-6 5 Pagliuca A, Williams H, Sailsbury J, Mufti GJ. Prodromal cutaneous lesions in adult T-cell leukaemia/lymphoma. Lancet 1990;335:733-4 6 Gessain A, Moulonguet I, Flageul B, et aL Cutaneous type of adult T-cell leukaemiallymphoma in a French West Indian woman. J Am Acad Dermatol 1990;5:994-1000 7 Dosaka N, Tanaka T, Miyachi Y, Imamura S, Kakizuka A. Examination of HTLV1 integration in the skin lesions of various type of adult T-cell leukaemia (ATL) independence of cutaneous type ATL confirmed by Souther blot analysis. Jlnvest Dermatol 1991;96:196-200 (Accepted 20 January 1992)
stage showed a similar but less severe pattern of ulceration of the colonic mucosa with bleeding. The haemoglobin was 5 g/dl and following transfusion treatment with prednisolone and sulphasalazine was commenced. A plain abdominal film demonstrated only minimal colonic dilation. Culture of the vaginal discharge demonstrated normal flora only. Two weeks later there was little improvement and a subtotal colectomy and right iliac fossa ileostomy was performed. Postoperatively the patient made a good recovery and the bleeding from the colonic vaginoplasty settled. The patient now remains well. Histological mination of the excised colon demonstrated extensive ulceration with pseudopolyp formation, mucin depletion and marked infiltration of the stroma characteristic of severe ulcerative colitis. The biopsies taken from the colonic vaginoplasty demonstrated similar changes with severe inflammation with ulceration of the colonic mucosa and granulation tissue formation. Discussion The bleeding from the colonic vaginoplasty may have occurred for several reasons. Firstly, it may be related to diversion colitis in which the macroscopic and microscopic features of ulcerative colitis occur in sections of colon and rectum following diversion of the faecal stream'. This arises because ofloos of luminal short chaiin fiday acids on which the colon depnd1fr a major part of its nutrition3-7. In his cae it seem- unlikely that diversion colitis would occur after 10 years ofisolation from the faecal stream. Secondly, it is possible that it is related to sepsis in the isolated section exacerbated by general debility associated with the extensive and severe ulcerative colitis but culture of the discharge from the neovagina in this patient did not demonstrate a specific pathogen. Thirdly, it is possible ulcerative colitis developed sronously in the colon and the colonic vaginoplasty with the changes in the neovagina improving, like the systemic manifestations of ulcerative colitis8, after colectomy. Only one other case report of ulcerative colitis developing in a colonic vaginoplasty has been identified9.
Case presented to Clinical Section 13 March 1992
0141-0768/92/ 070418-02402.00/0 o 1992 The Royal Society of Medicine
417
from neuroticed melanocytic nevi and schwanxomas. Arch
Dermaftl 1990;126:472-f6 10. Davidso SF, Das SK Srith,EE. Cellular schwannoma of the hand J4 Hand Surg [Am] 1988;14:907-9 .11 RabsdgSA Browse NL, Tighe JR, Fklher CD. Malignant nerve sheath tumour arising in a benign ancient schwannoma. Hietopathology 1989;14:525-8 12 Benzel EC, Morris DM, Fowler MR. Nerve sheath tumors of the sciatic nerve and sacral plexus. J Surg Oncol 1988;39:8-16
(Accepted 28 January 1992)
Chronic adult T-celIlkm ymphoma presenting with cutaneous mifestations
Case presented to Section of
Dermatology, 20 June 1991 24Kb w6L rw%nn 14Kb_ 9.5Kb_
W G Phillips MRCGP MRCP1 J A A Langtry BSc MRCP1 C Formstone BSc PhD2 A M R Taylor BSc PhD2 J R Marsden BSc MRCP1 1Department of Dermatology, The General Hospital, Steelhouse Lane, Birmingham B4 6NH and 2Department of Cancer Studies, Birmingham University, Birmingham B15 2TJ
'lif1rb,~)94I;
Keywords: adult T-cell leukaemia/lIymphoma; HTLV-1; poikiloderma
Mtow>Xelt.9K
b
2
3
I. Barn Hl beta chain ,Y' T5&9%Woior C C beta chain ;rn{;
Figwre2. (i-This Southern blot ofaBamHl digest ofDNA was probed ators for the T-ceLl receor C beta chain gene Lane us the DNMAshows the.germline band at 24 kg. Lane 2.shows DNA from
lymphocytosis.
a,T-cel
clone cutured in vitro. The germline band is lost and two
rearranged kqnde representing the two e iag lles of the
Case report A 67-year-old Afro-Carribean woman presented with an 8 month history of ger,eraized pruritus ndmalaise. She had been resident in the United Kingdom for 30 years and had no previous illness of note. There was no, history otblood transfusion, intravenous drug abuse or sexual activity with a high risk for acquiring WV., On examination she had widespread cutaneougs atrophy, telangiectasia, and reticulate hyperpigmentation characteristic of poikiloderma and widespread monomorphic dermal papules most marked on the face. There was 2-3 cm axillary, inguinal, and supraclavicular lymphadenopathy. There were no other abnormal physical findings, in particular there was
T-eUl C beta chain gene are present. Lane 3 shows DNA from the pdtients ymphoytes which show the germline band at24 kb and two rearranged bands at 14 kb and 26 kb showing thepresence ofa single T cel clone in peripheral blood. ( This Southern blot of an EcoRl digest of DNA- has been probed for HTLV-I. Lane 1 shows the inestigator DNA; The band at 8 kb represents a normal human sequenc and iua recognized artifact generated at the time of cloning the ITL V-i priobe. Lane 2 8hows DNA from the lymphoes of a paticet with GAuteATLL while laneS3shows DNA from our patients lymphocytes An extra band an b-seen in bbth hes2 and proing the presence of monoclonally integrated HTLV-I proviral DNA in both patients
no hepatomegaly or splenomegaly and there were no upper motor neurone signs in the lower limbs. Investigation revealed a lymphocytosis of 13x 1O9/1, 70% of which were CD3-ve CD4+ve s*Ua lymphqcytes. Skin -bopsy showed, an inffltra ofcytolpgically normal sa lnpho.ytea in te papi4lary derm wjrg h ei otropimd 'i. telkLymph nodeitgy h nsionoozmi4tent with T cell lymphomA. nne marrov , cpa
_
Z ..
'45
.
-
A
^. p
_
J
Figure 1. Biopry of poilod*ematous abdbmina shin *howingun infiltrate of cytologwally normal msnal lymphocytes gn he papikiry dermis with epiderotropism (H & E, xl1O00)
-
aspiate cytology showed- lymphoid infiltate comprising 40* of nuleated cello.,Soia of these cells had clQvOn nuaclei and 10% were immature. Serum cal a te dehydrgenase, skeletal a. and lymph te bromosopies were normal. Anti _HTLV-1 antibody iFas positive _y ELISA. T-cell receptor gene rearrangement s es ir dan oa al ToeU clonel is blood (ige 2). 4rder A show thatITLV l and te prec -of is related- to our patient's di anti-dHTLV-1 antibody was not an incidental finding we demoPtathe integration of HTLV-1 proviral DNA into the tumwourgenome. Ti was done by, Sothrn blotting an ECoR1 digest of DNA extracted from peripheral blood
Q14X-0768/92/ 070417rO2/$02.000/ i 1992 The Royal Society of Medicine
418
Journal of the Royal Society of Medicine Volume 85 July 1992
lymphocytes and probing for HTLV-1 (Figure 2). As there are no EcoRl restriction sites within the provirus1 and the provirus inserts at random within the genome of the hosts T-cells2 the presence of an extra band on this study shows that HTLV-1 proviral DNA is incorporated within the genome of a T-cell clone. Discussion
Monoclonal integration of the HTLV-1 proviral genome is associated with a spectrum of lymphoproliferative disease. Pre-ATLL is an asymptomatic lymphocytosis of < 10 000 without clinical evidence of organ infiltration3. Smouldering ATLL involves cutaneous infiltration without lymphocytosis. Chronic ATLL consists of lymphocytosis and cutaneous involvement without visceral disease. Acute ATLL is characterized by large numbers of leukaemic cells in peripheral blood, cutaneous, lymph node, bone and visceral involvement with poor prognosis4. Although cutaneous infiltration is a well recognized presentation of ATLL4-7 this is the first patient we are aware of with chronic ATLL who has presented with the combination of poikiloderma and leukaemic cells with the cytological appearance of normal small lymphocytes in peripheral blood.
Ulcerative colitis and bleeding from a colonic vaginoplasty
T W Hennigan FRCS N A Theodorou FRCS Department of Gastrointestinal Surgery, Charing Cross Hospital, Fulham Palace Road, London W6 8RF Keywords: ulcerative colitis; vaginoplasty; gender reassignment
Vaginal reconstruction using a segment of sigmoid colon is not a new procedure and is occasionally performed after gender reassignment surgery or following vaginal excision for carcinoma of the vagina. A case is presented in which such a patient developed ulcerative colitis in the colon and rectum and also developed similar inflammatory changes in the isolated section ofcolon in the vaginoplasty.
Case report A 26-year-old man had a gender reassigmnent 16 years ago having lived in a female role for 18 months. The patient developed a 'vaginal' prolapse which-was repaired twice but recurred. A vaginoplasty using approximately 12 cm of sigmoid colon was performed in 1980. In 1990, the patient presented with a 2 month history of rectal bleeding and diarrhoea with 10-15 bowel actions per day associated with colicky abdominal pain and weight loss of half a stone. In addition, a blood-stained 'vaginal' discharge had been noted. On examination the patient was pale with a tachycardia of 130 beatslminute but no pyrexia. The abdomen was distended with tenderness in the left iliac fossa. Sigmoidoscopy demonstrated contact bleeding and extensive ulceration. Examination of the neovagina at that Correspondence to: Mr T W HeDnigan, 125 Balfour Road, London W13 9TW
References 1 Yoshida M, Miyoshu I, Hinuma Y. Isolation and charcterisation of retrovirus from cell lines of human adult T-cell leukemia and its impcan in the diease. Proc Nati Acad Si USA 1989,7.2031-5 2 Seiki M, Eddy R, Shows TB, Yoshida M. Nonspecific integration ofthe HTLV1 provirus genome into Adult T-cell leukaemia cells. Nature 1984;309:640-2 3 Kinoshita K, Amagaski T, Ikeda, et aL Preleukemic state of adult T-cell leukemia: abnormal T lymphocytosis induced by human adult T-cell leukemia/lymphoma virus. Blood 1985;66:120-7 4 Kawano F, Yamaguchi K, Nishimura H, Tsuda H, Takatsuki K. Variation in the clinical course of Adult T-cell leukemia. Cancer 1985;66:851-6 5 Pagliuca A, Williams H, Sailsbury J, Mufti GJ. Prodromal cutaneous lesions in adult T-cell leukaemia/lymphoma. Lancet 1990;335:733-4 6 Gessain A, Moulonguet I, Flageul B, et aL Cutaneous type of adult T-cell leukaemiallymphoma in a French West Indian woman. J Am Acad Dermatol 1990;5:994-1000 7 Dosaka N, Tanaka T, Miyachi Y, Imamura S, Kakizuka A. Examination of HTLV1 integration in the skin lesions of various type of adult T-cell leukaemia (ATL) independence of cutaneous type ATL confirmed by Souther blot analysis. Jlnvest Dermatol 1991;96:196-200 (Accepted 20 January 1992)
stage showed a similar but less severe pattern of ulceration of the colonic mucosa with bleeding. The haemoglobin was 5 g/dl and following transfusion treatment with prednisolone and sulphasalazine was commenced. A plain abdominal film demonstrated only minimal colonic dilation. Culture of the vaginal discharge demonstrated normal flora only. Two weeks later there was little improvement and a subtotal colectomy and right iliac fossa ileostomy was performed. Postoperatively the patient made a good recovery and the bleeding from the colonic vaginoplasty settled. The patient now remains well. Histological mination of the excised colon demonstrated extensive ulceration with pseudopolyp formation, mucin depletion and marked infiltration of the stroma characteristic of severe ulcerative colitis. The biopsies taken from the colonic vaginoplasty demonstrated similar changes with severe inflammation with ulceration of the colonic mucosa and granulation tissue formation. Discussion The bleeding from the colonic vaginoplasty may have occurred for several reasons. Firstly, it may be related to diversion colitis in which the macroscopic and microscopic features of ulcerative colitis occur in sections of colon and rectum following diversion of the faecal stream'. This arises because ofloos of luminal short chaiin fiday acids on which the colon depnd1fr a major part of its nutrition3-7. In his cae it seem- unlikely that diversion colitis would occur after 10 years ofisolation from the faecal stream. Secondly, it is possible that it is related to sepsis in the isolated section exacerbated by general debility associated with the extensive and severe ulcerative colitis but culture of the discharge from the neovagina in this patient did not demonstrate a specific pathogen. Thirdly, it is possible ulcerative colitis developed sronously in the colon and the colonic vaginoplasty with the changes in the neovagina improving, like the systemic manifestations of ulcerative colitis8, after colectomy. Only one other case report of ulcerative colitis developing in a colonic vaginoplasty has been identified9.
Case presented to Clinical Section 13 March 1992
0141-0768/92/ 070418-02402.00/0 o 1992 The Royal Society of Medicine
