INTERNATIONAL JOURNAL OF LEPROSY^ Volume 58, Number 3Printed in the U.S.A.

An Unusual Case of Hansen's Disease(Lepromatous Leprosy) with CirculatingAnticoagulant and Macroglobulinemial

Rajendra G. Desai 2

A.B. is a 42-year-old Mexican laborer whoemigrated to the U.S.A. from Mexico 8 yearsprior to his presentation. He was in goodhealth until one year ago when he began tohave frequent coughs, colds, and minor nosebleeds. He was treated symptomatically. Hispast medical history included a diagnosisof neurofibromatosis made by the micro-scopic examination of biopsies of the nu-merous subcutaneous nodules he exhibitedthroughout his body.

On physical examination he had a stuffynose and an indurated ulceration of the hardpalate (2.0 x 3.0 cm) (Fig. 1). Multiple sub-cutaneous nodules were evident throughouthis body surface.

Laboratory evaluation disclosed a pro-longed activated partial thromboplastin time(APTT) of 51.3 sec (control = 33.1 sec). Hisprothrombin time and factor assays werenormal at 1/20 dilution (Table 2). Furtherexamination showed that the patient had animmediate inhibitor of APTT with low in-trinsic factor avidity which was readily di-luted. The discrepancy in APTT did notcorrect on mixing with normal plasma. Be-cause of the presence of abnormal circulat-ing anticoagulant, laboratory testing for lu-pus erythematosus was performed. HisANA, anti-DNA, and complement studieswere within normal limits. The patient hada negative VDRL test. A detailed drug his-tory was negative. The patient had an ele-vation of the gamma globulin fraction in apolyclonal distribution on serum proteinelectrophoresis (Table 1). Quantitative im-munoglobulin determination revealed anIgM of 1430 mg/d1. IgG and IgA were with-in normal limits. While the patient admit-

' Received for publication on 13 January 1989; ac-cepted for publication in revised form on 7 March1990.

R. G. Desai, M.D., Ph.D., 801 N. Tustin Avenue,Suite 404, Santa Ana, California 92705, U.S.A.

ted drinking 8 to 10 beers a week for severalyears, his liver function tests were normal.

The patient underwent biopsy of the sub-cutaneous nodules, nasal cartilage, and softpalate lesions without any bleeding com-plications. Pathological examination of thecartilage and palatal tissues initially showedchronic inflammation with a preponderanceof plasma cells, lymphocytes, and histio-cytes. The cells contained PAS-positive in-tranuclear globules ("Dutcher bodies")which are associated with IgM gammopa-thy. Zichl-Neelsen staining of the biopsymaterial from the subcutaneous nodules,however, gave a diagnosis of lepromatousleprosy (Figs. 2 and 3).

DISCUSSIONLeprosy (Hansen's disease) is an ancient

disease which is rarely encountered in theday-to-day practice of medicine in theU.S.A. However, it is becoming frequentlyrecognized in those areas where migrantpopulations from Mexico and South-EastAsia are prevalent. The presentation of si-nusitis, oral ulcer, and subcutaneous nod-ules in an immigrant patient should raisethe suspicion of Hansen's disease. Interest-ingly, unfamiliarity with this disease and

FIG. 1. Chronic ulcer, soft palate.

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58, 3^ Desai: Circulating Anticoagulant^ 463

TABLE 1.^Laboratory studies.

Hemoglobin 12.1 g%Hematocrit 35.6%White blood cells 6600/mm'Platelets 140,000/mm3Total protein 7.9 g%Albumin 3.8 g%Alpha-1 0.4 g%Alpha-2 0.9 g%Beta 0.7 g%Gamma 2.0 g%

Serum protein electrophoresis : poly-clonal gammopathy

IgG^ 1580 (650-1600 mg/ml)IgA^ 201 (100-400 mg/ml)IgM^1430 (18-280 mg/ml)24-hr urine^No paraprotein identifiedAntinuclear antibodies

(ANA)^NegativeAnti-DNA^NegativeC,^ 60 mg/d1C,^ 11 mg/dl

unawareness of its low infectivity rates cre-ated some anxiety in our medical personnel.The patient was eventually transferred tothe special unit for leprosy at the University

of Southern California Medical Center wheretreatment with rifampin and dapsone wasbegun.

An association of "lupus anticoagulant"with lepromatous leprosy has been previ-ously reported by Cole, et al. ( 2) in anasymptomatic patient without antecedenterythema nodosum leprosum. In Cole's case,the anticoagulant was felt to be an IgM im-munoglobulin. Basu, et al. ( 1 ) also reportedsignificant numbers of leprosy patients withan alteration in coagulation tests, but didnot screen them for circulating anticoagu-lant. It is suggested that screening coagu-lation tests should be performed in leprosypatients prior to surgical procedures. Ifscreening tests are abnormal, specific factorassays and a search for lupus anticoagulantmay be helpful.

SUMMARYA 42-year-old Mexican migrant laborer

with a previous history of neurofibroma-tosis presented with a stuffy nose and chron-ic ulceration of his soft palate. Multiple sub-cutaneous nodules were found on his skin,and laboratory investigation revealed an el-

TABLE 2. Coagulation tests.Patient^Control

Screening tests

time (sec)11.751.3

10.733.1

Prothrombin time (sec)Activated partial thromboplastin

Specific factor testsFactor VIII (VIII:C) 1/10 dilution 89% 50-170%

1/20 dilution 143%Factor IX 1/10 dilution 66% 50-170%

1/20 dilution 78%Factor XI 1/10 dilution 45% 50-150%

1/20 dilution 61%Factor XII 1/10 dilution 51% 50-150%

1/20 dilution 73%

Platelet aggregation testsRistocetin and collagin—NormalADP—HyporesponsivcEpinephrine—Normal primary

Weak secondary

APTT inhibitor screen^Normal 50%^Patient 50%Incubation^Patientat 37°C^ saline 50%^normal 50%

5 min^46.2^41.4^45.3^Positive for lupus-2 hr^ 42.0^52.4^like anticoagulant

464^ International Journal of Leprosy^ 1990

FIG. 3. Special Ziehl-Neelsen staining showingFIG. 2. Biopsy of subcutaneous nodule showing^clusters of leprosy bacilli.

lymphocytes, plasma cells and histiocytes.

evated activated partial thromboplastin time(APTT). Further laboratory evaluationshowed a lupus-like circulating anticoagu-lant deemed IgM by quantitative immu-noglobulin studies. Although coagulationdefects in lepromatous leprosy are rare, thepreoperative preparation of a patient withleprosy may require a screening prothrom-bin time (PT), APTT and platelet count.Abnormalities in these values may indicatethe need for specific factor assays and asearch for circulating anticoagulant.

RESUMENSe presenta el caso de un obrero de origen mexicano,

de 42 aims de edad, con una historia previa de neu-rofibromatosis, que presentO una nariz congestionaday ulccración crOnica del paladar blando. En su piel seencontraron multiples nOdulos subcutincos y los exd-menes de laboratorio revelaron un elevado tiempo par-cial de tromboplastina activada (TPTA) y la presenciade un anticoagulante circulante parecido al encontradoen lupus y de clase IgM, segün los estudios cuantita-tivos de inmunoglobulinas. Aunque los defectos de

coagulación son raros en Ia lepra lepromatosa, la pre-paración preoperativa de un paciente con lepra puederequerir de la medición del tiempo de protrombina(TP), del TPTA y del nemero de plaquetas. Las anor-malidades de estos valores pueden indicar la necesidadde ensayos para factores especificos y la büsqueda deanticoagulante circulante.

RESUMEUn travailleur emigre mexicain de 42 ans avec une

histoire antérieure de neurofibromatose s'est present&avec un nez encombre et une ulceration chronique dupalais. Des nodules sous-cutanés multiples furenttrouvés sur sa peau, et les examens de laboratoire ontmontré un temps de thromboplastine partielle activée(TTVA) eleve. La poursuite de ('investigation de la-boratoire montra des facteurs anticoagulants commedans lc lupus, que des etudes quantitatives ont revel&etre des IgM. Bien que des troubles de Ia coagulationsoient rares dans la lepre lépromateuse, la preparationpre-opératoire d'un patient atteint de lepre peut ne-cessiter la verification du temps de prothrombine (TP),et le comptage des plaquettes. Des anomalies dans cesvaleurs peuvent indiquer le besoin de dosages de fac-teurs specifiques et la recherche d'anticoagulants cir-culants.

58, 3^Desai: Circulating Anticoagulant^ 465

Acknowledgments. The author wishes to acknowl-edge the pathological diagnosis and special staining byK. Yamazaki, M.D., of Fountain Valley CommunityHospital, and the coagulation review of this paper byHerman Branson, M.D., Assistant Professor, Depart-ment of Pathology, University of California, Irvine.Special thanks to Anil deSilva, M.D., Albert EinsteinSchool of Medicine, for review of this manuscript. Thetechnological assistance of A. Fagin, M.D., and thephotographic help of Michael K. Samoszuk, M.D., aregreatly appreciated. S. Arya, M.D., referred this inter-esting patient.

REFERENCES1. BASU, A. K., CHATTERJEE, J. B., MUKHERJEE, A. and

GHOSH, S. Observations on hemostasis in leprosy.Bull. Calcutta Sch. Trop. Med. 18 (1970) 33-34.

2. COLE, F. S., BRUSCH, J. L. and TALARICO, L. Cir-culating anticoagulant in lepromatous leprosy. Int.J. Lepr. 47 (1979) 121-125.