Indian J. Pediat. 35 : 116, 1968

C H L O R O M A AS A PRESENTING FEATURE OF ACUTE LEUKAEMIA* Report of a Case with Brief Review

N. R. BHANDARIand O. P. [~AI)()NIA lndore

Chloroma is an unusual presenting feature of acute leukaemia in childhood. From the Greek "chloras" meaning green, the term refers to localized discoloured masses of leukemic tissue. These are most t o m monly found in relation~ to the periosteum of the skull, paranasal sinuses, orbits, vertebrae, ribs, etc. Since the first description in 1821 by Burns, many cases have been reported from all over the world. Huber first described the accompanying changes in the blood, but did not appreciate the significance of the association. The association with leukemia was first suspected by Von Recklinghausen in 1855. Recently Lusher ( 1 9 6 4 ) and Ross ( 1955 ) have also reported cases of leukemia with chloroma as a presenting feature. Classical chloroma consists of an orbital tumour frequently causing proptosis and exophthahnos in assoc;ation with, or even preccding, other symptoms and signs of acute leukemia. The onset may be insidious with gradual appearance of exophthalmos or swelling of the temporal region *From the Department of Pediatrics, M. G. M. Medical College, Indore.

eventually resulting in the chloromatous "froglike" facies. Of 336 eases reviewed by Edgerton (1947), 202 were under 15 years of age. A few cases have been reported in the neonatal period also ( Saxl 1937, Morrison et al. 1939). The typical tumour cells comprising a chloroma are primitive white blood cells, usually myeloblasts. The characteristic green colour is believed to be due to myeloperoxidase, an enzyme elaborated by these primitive cells. Rarity of the disease and paucity of case reports in the Indian literature (Sethi 1966) have prompted us to report this case.

Report of a Case

H. C., a 12-year-old male child, was admitted on 7th July, 1966, with the complaints of pain in the lower limbs, a purulent discharge from the left ear, asymmetry of the face and protrusion of the left eye for one month. There was no history of any significant pest infection. Labour and pregnancy were uneventful. The mother did no suffer from any infection during pregnancy nor was she exposed to radiation. The present illness started

BHANDARI AND B A D O N I A - - C H L O R O M A AS A PRESENTING FEATURE OF ACUTE LEUKAEMIA

2~ months back when the child developed fever associated with pain in the left ear and a purulent discharge. The temperature settled down .in 4 aays and the parents noticed deformity oftbe face on the left side. A month-and-a-half later, bulging of the left eye-ball was observed which was increasing. There was no history of headache, vomiting or convulsions. Physical examination revealed a thinly built boy weighing 75% of his expected weight, proptosis of both the eyes and a swelling lateral to the left orbit ( F i g . 1 ). There was slight weakness of the external rectus muscles on both sides. The temporal swelling measured 289 cm • 189 era., was firm, but not painful nor tender. The cornea and conjunctiva were clear. The external auditory meatus on the left side revealed the presence of a small-nodule 1 cm • 89 era., yellow in colour. The preaurieular glands were slightly enlarged and were mobile and tender. The cervical glands were not palpable. The inguinal glands on both sides were enlarged. The liver was not palpable. A few old small-pox marks were present on the body. The bones and joints were normal. There was a lower motor neurone type of facial paralysis on the left side. The fundus showed blurring of the disc margins and the vessels were congested. The rest of the examination did not reveal any abnormality. After I0 days' stay in the hospital the facial paralysis improved but the proptosis increased. There was no pus discharge. Glands all over the body became enlarged and were discrete and non-tender. The spleen became palpable. On admission, the total W . B . C . count was

I

17

41,O00/c.mm.

with 657o polys and 35~o lymphocytes. Haemoglobin was 8 G.70. No abnormal cells could be detected. After 15 days of admission, the total leucocyte count was 20,500/ c.mm., polys 6070, lymphocytes 3070 and immature white cells I07o. C. S. F. and skiagrams of the chest, extremities, skull and vertrebrae were normal. I.V. pyelography did not reveal anything abnormal. Biopsy of the nodule showed featules of adipose tissue with fibrous septa travelling through it, blood-vessels showing congestion and myeloblasts. Bone marrow and peripheral blood smear showed features of acute myeloid leukaemia. By this time there was bilateral proptosis, generalised lymphadenopathy and hepatosplenomegaly. During his stay in the hospital the child received six blood transfit,qons and general measures. "Endoxan" was given I.V. in doses of 250 mg. daily for five days followed by injections of 250 mg. each on alternate days after which 4 more biweekly injections were given. When the blood picture showed severe anaemia and leukopenia, blood transfusion was given. Corticosteroids in doses of 30 mg of prednisolone orally was started after the full course of Endoxan. After an initial improvement, the condition deteriorated and the child expired on 16.10.66. Autopsy could not be performed. Comment

This case of acute myeloid leukemia presented with chloroma. The child presented with bilateral proptosis and left-sided facial palsy, a retrobulbar mass and a nodule in the left ear long antedating any other evidence of acute leukemia. On admission the peripheral

118 INDIAN JOURNAL OF PEDIATRICS

VOL. 35 NO. 241

b l o o d picture did n o t s h o w a n y evidence o f acute l e o k e m i a but after a b o u t 15 d a y s ' time when b l o o d a n d bone m a r r o w were studied, a full fledged picture o f acute m y e l o i d leukemia was seen. D u r i n g this p e r i o d , the c o n d i t i o n o f the child went downhill. H e was k e p t o n the usual a n t i l e u k e m i c treatment and other supportive measures. After initial t r a n s i e n t i m p r o v e m e n t in the general c o n d i t i o n , the b l o o d p i c t u r e , a n d some r e c o v e r y o f left-sided facial paralysis, he d e t e r i o r a ted and died a b o u t 389 m o n t h s later. It is unusual f o r a case o f acute l e u k e m i a to present as c h l o r o m a with p r o p t o s i s . H o w e v e r , a n y child with p r o p t o s i s , unilateral o r bilateral, s h o u l d be investigated for evidence o f leukemia.. Summary

A 12-year-old-male child, p r e s e n t i n g with bilateral p r o p t o s i s a n d with features o f c h l o r o m a , later d e v e l o p i n g

a p i c t u r e o f full-fledged acute m y e l o i d l e u k a e m i a , is presented. T h e literature is briefly reviewed. We are indebted to Dr. K. M. Salgia, Department of Pathology, for studying the baematological aspects of the case. We are highly thankful to Dr. J. N. Pohowalla, Professor of Pediatrics, for valuable guidance and helpful criticism. Were also thankful to Dr. B. N. Jungalwalla, Dean and Dr. R. Bhattacharya, Medical Superintendent, for permission to use hospital records. References

Burns, Allen. Cited by Lusher. J. M. Edgerton, A. E. (1947). Chloroma. Trans. ,4met. Opthal. Soc. 45, 376. Gump, M. E., Hester, E. G. and Lohr, O. W. (1936). Monocytic chloroma. Arch. Opthal. 16, 931. Hubcr, K. Citcd by Lusher, J. M. Lusher, J. M. (1964). Chloroma and chloroleukemia. Amer. J. Med. 18, 671. Morrison, M. Samwixk, A. and Rubinstein, R. (1939). Congenital leukcmias with chloroma. Amer. J. Dis. Child. 58, 332. Ross, R. R. (1955). Chloroma and chloroleukcmia. Amer. J. Med. 18, 671. Saxi, O. Cited by Lusher, J. M. Sethi, A. S. (1966). Chlorc,ma. Indian J. Pedlar. 33, 208. Von Rccklinghausen, F. Cited by Lusher, L. M

ERRATUM

On page 442, line 7, December, 1967 issue read "-male" instead of "female."