Falkson, Geoffrey

Falkson, H.C.

Schulz, E.J.

English

AJOL - African Journals Online

2 January 1971 S.A. MEDICAL JOURNAL 19fascia' also suggests the diagnosis of rupture of a bron-chus.ComplicationsIf the patient survives and treatment is not prompt,three main complications may be expected.Atelectasis. The effects of atelectasis after successfulbronchial anastomosis have been studied by Mahaffeyet al.s Findings such as may be encountered in a right-to-left shunt are reported. They also noted a lower vitalcapacity, decreased oxygen uptake and a decreased pul-monary compliance.Bronchial stenosis. There is no uniformity of thoughtas to the frequency with which stenosis will result froman untreated fractured bronchus.",,5,7,9-11Pulmonary sepsis. This is the most severe complication.Although Drapanas et al.'· report a case of completereversal of bronchiectasis after re-anastomosing the twobronchial ends, it is felt that this is the exception ratherthan the rule. Coxatto and Lanari," in their study of thepathogenesis of bronchiectasis, feel that where there iscomplete obstruction to the distal bronchus, bronchialsecretion will cease before irreparable damage has beendone because of a pressure build-up due to bronchialsecretions.Mahaffey et al.' reported a case of complete recoveryafter anastomosis was done, where only a sterile gelati-nous plug was aspirated from the distal segment. If, how-ever, secondary infection develops, permanent damage isalmost inevitable. This unhappy finding occurs in about25% of cases, mostly those where the obstruction is in-complete.13TreatmentAlthough cases of successful repair of ruptured bronchihave been reported as long as 11 years after the fracturehad occurred," the treatment of choice is immediate re-anastomosis of the fracture, or suturing of the lacerationwith interrupted black silk or wire sutures.SUMMARYTwo cases of fractured bronchi which have been successfullytreated have been reported. Traumatic fractures of the bronchifollowing blunt chest trauma have been discussed in generaland the importance of early repair to prevent stenosis and/orpermanent lung damage has been stressed again.REFERENCES1. Burke, J. F. (1962): J. Amer. Med. Assoc., 181, 683.2. Hood, R. M. and Sloan, H. E. (1959): 1. Thorac. Cardiovasc. Surg.,38, 458.3. Krinitsky, S. t. (1927): Virchows Arch. path. Anal., 266, 815.4. Griffith, J. L. (1949): Thorax, 4, 105.5. Ballershy, J. S. and Jilman, J. W. (1964): Arch. Surg., 88, 644.6. Marchand, P. (195t): Thorax, 6, 359.7. Adarns, W. E. (1966): J. Amer. Med. Assoc., 198, 299.8. MahafIey. D. E., Oscar, C. jnr, Hollis, G. B. and DeBakey, M. E.(1956): J. Thorac. Surg., 32, 312.9. Winter, B. and Baum, R. (1968): J. Amer. Med. Assoc., 206, 370.10. Drapanas, T., Siewers, R. and Feist, J. H. (1966): New Engl. J. Med.,275, 917.11. Ozonoff, M. B. (1964): Cali£. Med.. 100. 14.12. Coxatta, O. C. and Lanari, A. (1954): J. Thorac. SUTg., 27, 5t4.13. Reynolds, J. and Christensen, E. E. (1968): Tex. SI. J. Med., 64,50.PORPHYRIA IN HODGKIN'S DISEASE*H. C. FALKSON, M.D., Department of Cancer Chemotherapy, E. J. SCHULZ, M.MED. (DERM.), Department ofDermatology, AND GEOFFREY FALKSON, M.D., Department of Cancer Chemotherapy, H. F. Verwoerd Hospital andUniversity of Pretoria, PretoriaVaried and perplexing skin lesions are found in thecourse of Hodgkin's disease. A survey of the worldliterature shows that 20 - 50% of patients have significantcomplaints referable to the skin; no cases with concomi-tant porphyria have as yet been reported.Although porphyrin was referred to and described asfar back as the last century'" and the term porphyria wasfirst used by Waldenstrom' more than 30 years ago, manyaspects of this enigmatic condition still await clarification.Indeed, even the classification of porphyrias must awaitmore knowledge regarding their inter-relationships, andis constantly being revised. The most useful currentcIassificati0n is shown in Table I!The following case report draws attention to a hithertounpublished concomitant metabolic disorder occurringduring the course of Hodgkin's dis::ase.CASE REPORTA 39-year-old diamond cutter was referred to our clinicwith the diagnosis of Hodgkin's disease of mixed celltype (Luke's classification). Biopsies were performed onan enlarged lymph gland in the left inguinal area andkin from the pubic area. About 4 months previously hestarte~ complaining of pruritus, weight loss, fever andsweatmg.When the patient was first seen in our clinic he was'Date received: 28 August 1970.TABLE 1. CLASSIFlCAHON OF PORPHYRtAS (1970)'1. Erythropoietic porphyria(a) recessive (classical congenital porphyria)(b) dominant (porphyria erychropoiecica et hepacica or-erythropoietic protoporphyria or coproporphyria)2. Acute intermittent porphyria(a) manifest(b) latent3. Hepatic cutaneous porphyria(a) hereditary(i) porphyria cutanea tarda or protocoproporphyria(ii) mixed porphyria(iii) variegate porphyria(b) acquired(i) symptomatic porphyria cutanea tarda(ii) Bantu porphyria(iii) hexachlorobenzene porphyria(iv) griseofulvin porphyria(v) porphyria of hepatic adenoma4. Experimental porphyriafound to be very ill with a deep jaundice, severe nausea..and pain over the right hypochondrium and lower back.He further complained of loss of appetite and a moderate'dead feeling' of all fingers. Examination showed hilateralmultiple enlarged lymph glands of the groins as well asa large, hard swelling of the anterior pubic area, andhepatomegaly. A severe ichthyosis of the whole bodywas present.The following laboratory results were obtained at this.20 S.-A. MEDIESE TYDSKRIF 2 lanuarie 1971time: Haemoglobin 13·5 g/ 100 ml, platelets 229000/ mm'.white cells 25000/mm3 with a differential count of 90%polymorphonuclear cells, 4% Iymphocytes and 6% mono-cytes. The Coombs test was negative, and a bone-marrow'examination showed no abnormalities. Blood urea was 34mg/ 100 ml, uric acid was 3·8 mg/ 100 ml, creatinine was 1·5mg/100 ml, serum calcium was 10·6 mg/ 100 ml, and serum'copper was 190 p.g/ 100 ml. The sedimentation rate was 40mm (Westergren), bilirubin direct/indirect was 1'5/0'8 mg/100 ml, SGOT was 23 Cabaud units, alkaline phosphatase32 KA units, serum albumin 2·6 g/100 ml, serum globu-lin 4·7 g/100 ml and serum gammaglobulin 1·5 g/100 ml.A marked bilirubinuria was present.Intensive combination chemotherapy was started imme-'<liately. By the 8th day of treatment the liver was nolonger palpable, the adenopathy was very much smallerand the pubic swelling half the initial size. Both pruritusand pain had cleared, and the generalized ichthyosis wasonly mild. The patient's appetite improved. The icteruswas only mild, sedimentation rate was down to 10 mm,bilirubin was normal, SGOT was 12 Cabaud units, serumalbumin 3·3 g/ 100 ml, serum globulin 4·2 g/lOO ml, alka-line phosphatase 22 KA units. Serum copper was still200 p.g/lOO ml.By the 28th day the jaundice had cleared, there wasno bilirubinuria, serum albumin was 3·7 g/l00 ml andserum globulin 3·3 g/l00 m!. Alkaline phosphatase wasstill above normal (20 KA units). Serum copper was162 p.g/lOO m\. Serum electrophoresis remained normaland the alkaline phosphatase gradually became normal.An occasional abnormal value was still periodically re-corded for SGOT and SGPT tests. By day 169 all testsindicative of liver function were normal, and they haveremained so up till the present time. On day 176 thepatient complained of blisters on both hands. He wasfound to have several large (up to 1·5 cm by 0·5 cm)subepidermal bullae, mainly on the dorsae of both hands(Figs. I and 2). These bullae contained a colourless fluid,and in other areas the blisters were broken and crustedover. No bullae occurred on the rest of the body.Urine and faeces were positive for porphyrins. Quanti-tative and qualitative tests were performed. The faecesshowed an increased coproporphyria of 50·86 p.g/ g dryFig. 1. The typical appearance of the patient's hands. Alarge unruptured bulla can be seen on the ring finger ofthe right hand.Fig. 2. Scarring and depigmentation as well as crusted,ruptured bullae are clearly visible on the dorsae of bothhands.faeces, and an increase of protoporphyrins to 71·87 p.g/ gdry faeces. No uroporphyrins were present. In the urine thecoproporphyrins were greatly increased to 152·66 p.gjIitreurine, while no uroporphyrins were found.The patient was in complete remission from the Hodg-kin's disease 170 days after treatment was started and isstill in complete remission 10 months after the commence-ment of treatment. The cutaneous porphyria has remainedpresent, which is rather irksome in his work.DISCUSSIONIn this patient several skin lesions occurred: specificallyHodgkin's disease of the skin (as was shown by thebiopsy of the pubic area), the non-specific ichthyosis ofHodgkin's disease (which cleared on successful treatmentof the Hodgkin's disease) and lastly, an unusual concomi-tant porphyria with typical skin lesions. When the por-phyria presented shortly after grossly abnormal liverfunction was restored, it was thought to be an acquiredsymptomatic form of porphyria cutanea tarda based onmalfunction of the liver. An interesting aetiological as-pect observed by Gajdos-Torok' is the rather frequentappearance of cutaneous porphyria in patients with rarediseases of haematological character. This could also fitin with our patient. The age and sex of this patient,absence of serious photosensitivity, no family history ofporphyria, the fact that administration of barbiturateshad had no adverse effects on him, and of course theacquired liver malfunction, suggested a symptomatic por-phyria cutanea tarda. Differentiation between symptomaticporphyria and genetic porphyria on purely clinical groundsmay be impossible."'; Quantitative and qualitative bio-chemical findings in the excreta (increased copro- andprotoporphyrins with absence of uroporphyrins) indicatethat this patient probably has a porphyria cutanea tardahereditaria (protocoproporphyria of Waldenstrom) orelse the so-called 'variegate' porphyria of White SoutbAfricans.The aetiological mechanism for the development ofporphyria awaits clarification. An elevation of the por-phyrins or their precursors in urine and faeces is thechemical hallmark of the porphyrias; this may be causedeither by a block in haem biosynthesis or, more likely.-2 January 1971 S.A. MEDICAL JOURNAL 21REFERENCES.1. Seherer, J. (184t): Annals of Chemical Pharmacology, 40. 1.2. Thudicum. 1. L. (1867): 10th Report of the Medical Officer of rhePrivy Council. London: H.~1. Stationery Office.3. Wa]denstrom. J. (1937): Acta med. scand .. suppl. 82.4. Gray, C. H. in Thompson. R. H. S. and WOOttOD. 1. D. P•. eds.(1970): Biochemical Disorders in Human Disease, pp. 215 - 232.London: J. & A. Churchill.5. Gajdos. A. and Gajdos-Torok, M. (1963): S. Afr. J. Lab. Clin. Med ..9, 295.6. Eales, L. (1960): Ibid., 6. 63.7. Eales, L.. Dowdle, E. B.. Levey, M. J. and Sweeney, G. D. (1966):S. Afr. Med. J., 40, 380.8. Ratner, A. C. and Dobson, R. L. (]964): Arch. Derm.. 89, 505.9. Busman. G. J. and Johnston, J. M. (1943): Penn. Med. J., 46. 1153.10. Levin, O. L. and Behrman. H. T. (1944): J. Mt Sinai Hosp., H, 207.11. Pillsbury, D. M. and Caro, W. A. (1966): Med. Clin. '. Amer., 50,1295.]2. FindJay. G. H. (1963): S. Afr. J. Lab. Clin. Meu .. 9. 162.SUM~IARYSkin lesions are very common in Hodgkin's disease, but nocases with concomitant porphyria have as yet been reported.A case is presented where protocoproporphyria with typicalskin involvement occurred when the patient with advancedHodgkin's disease. and severe liver involvement was in com-plete remission. Biochemical difIerentiation between sympto-matic porphyria cutanea tarda and genetic porphyria showedthat this patient has the latter form. The possi ble inter-relationship between Hodgkin's disease and the metabolicdefect of porphyrin metabolism is discussed.We wish to thank Dr J. L. Meiring, pathologist. for thequantitative and qualitative porphyrin determinations.maintenance therapy for I week before the appearanceof the bullae.Chlorambucil (N- '-dil2-chloroethyl)-p-amino-phenyl-bu-tyric acid) is one of the oldest alkylating agents and hasbeen in clinical use since 1954. It has been given forlong periods in a vast number of patients throughout theworld, and has never precipitated an attack of porphyria.This may perhap be because the incidence of porphyriais only 0'2 uu" or 0 07°u for outh African genetic por-phyria.by increased production of porphyrin precursors. Investi-gations suggest that increased amounts of glycine or delta-;minolaevulinic acid are available for porphyrin biosyn-thesis. Since S-aminolaevulinic acid is a common precur-sor for both porphyrin and purine biosynthesis, it hasbeen suspected that an abberation in purine metabolismmay represent part of the hepatic porphyrias. Since thefirst symptoms of cutaneous porphyria the patient's serumuric acid has remained elevated, ranging from 7·5 to 8·8mg/100 m!. The coexistence of porphyria and hyperuri-caemia may seem paradoxical, if a block in the manufac-ture of purines is a defect in porphyria. However, meta-bolic defects like gout and porphyria are not incompatible;and the presence of hyperuricaemia does not necessarilynegate the possibility that a block exists in the formationof purines.The case reported here presents a complex problemregarding the aetiology of the porphyria: (l) this may bea patient with porphyria variegata, totally unrelated tothe Hodgkin's disease; (h) the porphyria may be partof the Hodgkin's disease-although concomitant porphy-ria and Hodgkin's disease have never before been docu-mented, id reactions of bullae and vesicles during thecourse of Hodgkin's disease have been reported;'1O butthe cases were not investigated for porphyria (hydroaaestivale is not at all uncommon in Hodgkin's disease,and it has been noted" that some cases of so-called hydroaaestivale or polymorphous light eruption may well be"due to porphyria); (iii) lastly, the question arises whethera chemotherapeutic agent may have been a precipitatingfactor in a patient with a genetic defect. The porphyriaoccurred when the patient was already in complete re-mission with normal liver function; 3 weeks had elapsedsince intensive combination chemotherapy had beenstopped; and he had been receiving chlorambucil asPASSING EVENTS : IN DIE VERBYGAAN**Drr. J. H. T. ReYl/eke en F. J. Z. Booysen, interniste. praktiseervanaf 1 Januarie 1971 in vennootskap te Listergebou 907,Jeppestraat 195, Johannesburg. Telefoonnommers: spreek-kamers 23-1498, 23-2346.**University of the Wirwatersrand Medical Graduates Associa-liol/ Luncheon Club. The next meeting will be held on Tuesday12 January at 1.00 p.m. in the Blue Room, Station Concourse,Johannesburg. Miss M. Bryant, Public Relations Officer, Jo-hannesburg Stock Ex.change, will speak on 'The function ofthe stock exchange'. The cost of the luncheon is R1.55 perhead,. a~d all interested persons are welcome to attend.Application should be made to the Secretary, Medical Gradu-ates ASSOCiatIOn, Medical School, Hospital Street, Johannes-burg (telephone 724-6456).Universiteil van Slellenbosch, Fakulleil van Geneeskunde, Op- l/llernarional Agency for Research on Cal/cer, Researchkl/appingskursus in Radiologie vir Mediese Praktisyns. 'n .' Training Fellowships 1971 - 1972. Applications for trainingOpknappingskursus in radiologie, aangebied deur die Fakulteit 'fellowships in 1971 - 1972 are invited from junior scientistsvan Geneeskunde van die Universiteit van Stellenbosch, sal wishing to be trained in any aspect of laboratory and clinicalgehou word op 16 en 17 Januarie'1971 in die Kart Breine~.: .., cancer research. The Agency is especially interested in re-hospitaal, Bellville, K.P. Die kursus volg op vorige kursusse iiJ ceiving applications for training in epidemiology, biostatisticsradiologie en interne geneeskunde wat sedert 1968 aangebied is. and all aspects of environmental biology. Applicants shouldHierdie opknappingskursus handel oor verskeie aspekte van be engaged in research in medical or allied sciences and intendradiologiese diagnose en radiologiese behandeling in verband to pursue a career in cancer research. .met kanker, tuberkulose, artritis, vrouesiektes, amebiase en Fellowships are awarded for I year and are tenable in abilharzia. Daar sal ook besoek gebring word aan die radio- suitable institution abroad. Fellows will, in general, be selectedterapeutiese en radiodiagnostiese afdelings van die hospitaal. froin applicants with less than 5 years' postgraduate experienceDie kursus strek oor It dag vanaf Vrydagoggend om 9.00' Xrn. in medicine or the natural sciences. They must have an ade-tot Saterdag om 12.00 middag, en vind plaas onder die leidmg quate knowledge, both written and spoken, of the languagevan prof. C. J. B. Muller, hoogleraar in radiologie. Die lesings of the country in which their fellowship is tenable.word deur lede van die personeel van hierdie departement ge- Applications cannot normally be accepted from peoplelewer. ' already holding fellowships enabling them to study abroad.Inskrywing kan tot 8 Januarie 1971 gedoen word en navrae Stipends will vary according to the cost of living in theen inskrywings kan gerig word aan die Sekretaris, Opknappings- country of study. The cost of travel for the applicant and.kursus in Radiologie, Fakulteit van Geneeskunde, Posbus 53, in certain circumstances, that of his wife and children, willBellville, K.P. (telefoon: 97-5761). be met.Fellowship application forms and more detailed informationare available from the Chief of the Research Training andLiaison Unit, International Agency for Research on Cancer,16, Avenue Marechal-Foch, 69 - Lyon (6 E ) France. Applica-tions must reach the Agency not later than 28 February 1971.

Porphyria in Hodgkin's disease

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Porphyria in Hodgkin's disease

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