Case Reports

Indian Pediatrics 1999;36: 925-927

Hodgkin's Disease Presenting with Bone Involvement

From the Departments of Pediatrics and Hemato logy*, Safdarjang Hospital, New Delhi 110 029, India.
Reprint requests: Dr. Rajesh Mehta, Pediatrician, Department of Pediatrics, Safdarjang Hospital, New Delhi 110 029, India.
Manuscript Received: August 18, 1998;
Initial review completed: December 12, 1998;
Revision Accepted: April 5, 1999

Hodgkin's disease presenting with bony lesion is extremely unusual. About seventeen such cases have been reported in the literature, majority being in adults and adolescents(1). There is only one case report from India so far(2). Such cases prove to be a diagnostic dilemma and the resultant delay in diagnosis leads to poor prognosis. We present one such case in a child.

Case Report

A ten-year-old boy, resident of Western Uttar Pradesh, presented with the complaints of irregular fever and progressively increasing pallor for three months. He also complained of pain in the left thigh for fifteen days. The pain was dull in character and was moderately intense without any radiating character. He gave a history of general weakness, lethargy, anorexia and weight loss. There was no history of any bleeding manifestation, respira tory, urinary or gastro-intestinal symptoms. The child had received six blood transfusions before admission to this hospital. No other definitive treatment was given.

On examination the child was found to be cachectic and severely pale. There was no significant lymphadenopathy. There were no petechiae or ecchymoses and sternal tenderness was absent. On abdominal examination spleen was enlarged 8 cm and liver was palpable only 2 cm with normal consistency. There was no other lump or free fluid in the abdomen. On local examination the left upper thigh was tender on deep palpation but there was no obvious swelling or induration. Res-piratory and cardiovascular systems were normal.

Investigations revealed a hemoglobin of 3.5 g/dl, total leukocyte count of 7200 per cu mm with normal differential count, reticulocyte count of 1.2% and platelet count of 1,50,000 per cu mm. Peripheral smear showed a normocytic, normochromic blood picture and was otherwise unremarkable. X-ray chest was within normal limits. X-ray of pelvis with hip joints revealed an infiltrative osteolytic lesion of the right ischium with destruction of the cortical margin. The upper one-third of shaft of the left femur revealed symmetrical onion peel periosteal reaction with normal underlying cortex and medulla. Rest of the skeletal survey revealed no other bony involvement. Ultrasound abdomen showed a normal liver, moderate splenomegaly with normal echotexture and no other abnormality. Bone marrow aspiration from right postero-superior iliac spine was unremarkable and no parasites were seen.

An open surgical biopsy was performed from the left femur which showed a pleo-morphic infiltrate of polymorphs, eosinophils, histiocytes and plasma cells. The impression given by the histopathologist was that of eosinophilic granuloma.

In the meanwhile the patient received supportive care in the form of blood transfusion. A bone marrow biopsy was performed from the iliac crest. The impression smears revealed a normocellular marrow with un- remarkable erythroid, myeloid and mega-karyotic series. The finding of mononuclear Hodgkin cell and an occasional Reid-Sternberg cell clinched the diagnosis (Fig. 1). A final diagnosis of extranodal Hodgkin's Lymphoma was made and the patient was put on 6 cycles of COPP regime. The child achieved complete clinical and hematological remission. His spleen regressed, the hemo-globin stabilized and the left femur has shown radiological resolution. The pain and tenderness subsided and there was no functional deficit in the left leg.

Fig. 1.Touch preparation from bone marrow biopsy showing Hodgkin cell (Leishman stain).

Discussion

Hodgkin's disease usually presents with nodal and visceral enlargement. Bone involvement in Hodgkin's Disease at the time of presentation is extremely rare(3,4). Kaplan et al.(5) reported only four cases out of 334 cases of Hodgkin's Disease to have radiographically demonstrable skeletal involvement at the time of initial diagnosis. Gross et al.(1) reported 2 cases of primary Hodgkins disease of bone. Gall and Mallory(6) did not find a single case with initial bone involvement out of 229 cases of Hodgkin's disease studied by them. Most of the cases with bone involvement at the initial presentation have been adolescents and a few adults. Ours is apparently the only Pediatric case in the literature so far.

Osseous involvement in Hodgkin's disease occurs through hematogenous spread or direct spread from the contiguous involved lymph node. The presenting compalints of bone involvement are usually insidious onset of pain with associated weight loss and malaise. Radiologic evaluation reveals osteo-sclerosis alone, osteolysis alone or a combination of the two. Osteolytic lesions are poorly defined and are associated with periostitis in approximately one-third of the cases(7). Technitium-99 bone scan may disclose other clinically inapparent sites of bone involvement. The common sites are spine, pelvis, ribs, femur bones and sternum. The histologic picture is often confusing. The infilteration is pleomorphic with a mixture of plasma cells, lymphocytes, eosinophils, neutrophils, histiocytes and endothelial cells. In a series of 17 cases(8) the initial histological diagnosis was variously reported as Paget's disease, metastatic tumor, chronic osteomyelitis, primary sarcoma and eosinophilic granuloma (like in the present case).

For isolated bone lesions radiotherapy is quite effective and long term disease free survival in such patients has been reported(9). In the present case, there was bone marrow involvement, therefore, chemotherapy was resorted to with good result.

References

1. Gross SB, Robertson Jr WW, Lange BJ, Bunin NJ, Drummond DS. Primary Hodgkins disease of Bone. A report of two cases in adolescents and review of the literature. Clin Orth Rel Res 1992; 283: 276-280.

2. Sandhu MS, Biswal BM, Sharma MC, Karak AK. Primary Hodgkin's disease of bone: Report of a case. Indian J Med Ped Oncol 1995; 16: 32-35.

3. Levanthal BG, Donaldson SS. Hodgkin's Disease. In: Principles and Practice of Pediatric Oncology. Eds. Pizzo PA, Poplack DG. Philadelphia, Lippincott, 1989; pp 457-476.

4. Bonnadonna C, Wiermik PH, Santoro A. Clinicl treatment of Hodgkins disease. In: Neoplastic Diseases of the Blood, 2nd edn. Eds. Wiermick PH, Cannelos GP, Kyte RA, Schiffler CA. Edinburg, Churchill Livingstone Inc, 1991; pp 701-772.

5. Kaplan HS. Hodgkin's Disease, 2nd edn. Cambridge, Harvard University Press, 1980, p. 220.

6. Gall AE, Mallory TB. Management of lymphoma. Am J Pathol 1941; 18: 381-415.

7. Resnick D, Haghighi P, Myeloproliferative Disease. In: Bone and Joint Imaging, 2nd edn. Ed. Resnick D. Philadelphia, W.B. Saunders Co, 1996; pp 2247-2294.

8. Chan K, Miller DR, Jan C. Hodgkin's disease in adolescents presenting as a primary bone lesion. A report of four cases and review of literature. Am J Pediatr Hematol-Oncol 1982; 4: 11-17.

9. Devita VT, Manch PM, Harris NL. Hodgkin's disease. In: Cancer: Principles and Practice of Oncology, 5th edn. Eds. Devita VT, Hellman S, Rosenberg SA. Philadelphia, Lippincott Raven, 1997; pp 224-2283.