Minimal Change
Disease and Hodgkin's Disease: A Rare Association |
Ajay P. Sharma
Sanjeev Gulati
Rakehs Pandey*
From the Departments of Nephrology and Pathology*, San jay Gandhi Post Graduate Institute of Medical Sciences, Lucknow 226 014, India.
Reprint requests: Dr. Sanjeev Gulati, Department of Nephrology, San jay Gandhi Post Graduate Institute of Medical Sciences, Lucknow 226 014, India.
Manuscript received: November 5, 1998; Initial review completed: December 9, 1998; Revision accepted: January 13, 1999.
Children with nephrotic syndrome can have cervical lymphadenopathy due to various etiologies including local throat infection, tuberculosis and various lymphoproliferative disorders. Although lymphoma is an uncommon cause of cervical lymph- adenopathy yet it is important to suspect it, as its diagnosis at early stage has got both therapeutic and prognostic significance. The association between Hodgkin's disease (HD) and minimal change disease (MCD) though well known(1,3) is uncommon in children(3). A review of literature revealed 45 cases of HD associated with MCD and only four of these were from pediatric population(1,4,6). We report here a child with this rare association of HD and MCD.
Case Report
An eleven-year-old child was referred to our hospital with bilateral
cervical lymphadenopathy since last 2 years and anasarca since last 10 months. He was having persistent eosinophilia
since the time it was detected first time 6 months ago. Before being
referred, he received antitubercular treatment for one month emperically for persisient cervicallymphadenopathy, diethyl-carbamazine for persistent eosinophilia and for nephrotic syndrome he did not receive any steroids. At admission his physical examination revealed firm, nontender hepatomegaly, splenomegaly, firm nontender nonmatted
generalized lymphadenopathy and edema feet. Laboratory investigations revealed hemoglobin of 9.2 g/dl, white blood cells (WBC) count of 5200/cu mm with eosinophil counts of 16%, blood urea nitrogen of 10.9 mg/dl, serum creatinine of 0.7 mg/dl, serum protein of 3.7 g/dl, serum albumin of 1.1 g/dl, blood choles- terol of 449 mg/dl, 24 hour urine protein of 3.78 g with no red blood cells and white blood cells and urine analysis. Abdominal ultrasound revealed hepatomegaly, splenomegaly with normal sized kidney and no retroperitoneallymphadenopathy. Renal biopsy showed features suggestive of minimal change disease (MCD). Cervical lymphnode biopsy showed aggregates of epitheloid histocytes associated with multinuclear histocytic giant cells, and foci of infiltration with mature lymphocytes, eosinophils, polymorphs, plasma
cells containing prominent nucleoli, suggestive of mixed cellularity type of HD. Ziehl Neelsen stain for acid fast bacilli was negative. On staging, HD was found to be stage III. He was advised treatment for lymphoma but it was refused due to financial reasons, therefore he was discharged.
Discussion
The association between HD and nephrotic syndrome (NS) is well established( 1,3). In a review comprising 1700 cases of HD only 7 patients with MCD were found(3), showing the rarity of this association. MCD
has been reported to be the commonest renal lesion in nephrotics with HD(1,3). The mixed cellularity type of HD
was found to be more predisposed to development of MCD(7,8). In our patient also, HD was found to be of mixed cellularity type and kidney biopsy revealed MCD.
Different reports have shown variability in interval between onset of HD and MCD.
The four cases reported in pediatric age group also showed this
variation. In two patients HD preceded NS by period of 12 and 108
months. In other two patients HD followed NS by an interval of 10
and 18 months. Different mechanisms have been put forward to explain the association between HD and
MCD. The most acceptable one is that there is disorder of T-cell functions. However, the exact pathogenesis is still not clear. Despite coexistance of lymphadenopathy and anasarca for last 10 months, HD was not diagnosed in the index case. On the contrary, the patient was emperically treated for worm in- festations, tropical eosinophilia and tuberculosis, whereas the eosinophilia and lymphadenopathy were due to HD, as con- firmed by lymph node biopsy. In this part of the world, tuberculosis isa common infection in children(9) in NS. The prevalence of lymphoma in this part of country has been reported to be similar as in the rest of the world (lO). On the other hand nephrotic syndrome
has been reported to be more common in Asians( 11), still this
association of HD and NS has not been reported from India. This may
be due to poor reporting or probably due to under recognition. The
early recognition of this association is crucial as NS associated
with lymphoma has entirely different management and an early diagnosis can help in improving the prognosis. Though the association of MCD and HD is quite rare, yet it should be kept in mind, especially in children presenting with anasarca who have associated eosinophilia and/or lymphadenopathy. This would avoid delay in diagnosis of lymphoma and over treatment for tuberculosis.
To conclude, although NS and HD is a rare association, HD should be considered in a patient of nephrotic syndrome with lymphadenopathy and histopathological
examination of the enlarged lymph nodes should be performed instead of emperical antitubercular therapy.
Acknowledgement
The authors appreciate the secretarial asistance of Mr. Santosh Kumar Verma.
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