Hodgkin Lymphoma With Central Nervous System Disease at Primary Presentation

Letter to Editor: Pediatric Hodgkin lymphoma (HL) rarely presents with central nervous system (CNS) involvement at the time of initial presentation. Rather, it is more often seen in patients who have relapsed. We report a child with HL who had CNS involvement at initial presentation. A 6-year-old female presented with complaints of fever for 5 months, jaundice, and multiple nodular swellings in the neck and axilla for 3 months. On physical examination, she was febrile, pale, and icteric with cervical and axillary lymphadenopathy with largest lymph node 2 × 2 cm and massive hepatosplenomegaly. Laboratory investigations revealed anemia (hemoglobin 8.8 g/dl), total leucocyte count 5.2 × 109/l, platelet count 256 × 109/l, and abnormal liver function tests. Fine-needle aspiration cytology from the axillary node revealed scattered histiocytic clusters and atypical cells, with high nuclear–cytoplasmic ratio, hyperchromatic nuclei, and small nucleoli and the same was confirmed on biopsy. Immunohistochemistry displayed CD15 and CD30 positivity, which was consistent with the diagnosis of Hodgkin lymphoma, mixed cellularity type. Bone marrow biopsy showed infiltration with the tumor cells. Contrastenhanced computed tomography scan of the chest and abdomen revealed mediastinal and mesenteric lymphadenopathy along with hepatosplenomegaly. Considering Hodgkin lymphoma stage IVB she was started on gemcitabine, dexamethasone, cisplatin (GDP) chemotherapy regimen. On day 2 of chemotherapy, she had multiple episodes of generalized tonic clonic seizures that were associated with transient loss of vision in the postictal phase. Fundus and neurological examination remained normal and the patient child was managed with antiepileptics. Magnetic resonance imaging of the brain revealed multiple leptomeningeal deposits in the parieto-occipital and frontal region with altered signal intensity in corresponding regions (Fig. 1). Cerebrospinal fluid revealed pleocytosis with Reed–Sternberg cells. Chemotherapy was stopped, suspecting gemcitabine to be causative agent for loss of vision. She regained her vision after 5 days and remained seizure free. Thereafter, the chemotherapy protocol was changed. Radiotherapy was planned for CNS disease, but due to financial constraints, the child left against medical advice and could not be followed up. Hodgkin lymphoma involving the brain or meninges is uncommon and is usually seen in patients with uncontrolled systemic disease or with relapse.[1–5]. The median interval from initial diagnosis to intracranial disease presentation is almost 47 months.[2] Mostly, CNS HL shows a supratentorial spread, manifesting as cranial nerve palsies, motor or sensory deficits, headache, papilledema, coma, or seizures.[2,6] This case report highlights a rare clinical situation where a child developed CNS involvement in HL at the time of presentation. There are no definite guidelines for the management of CNSHL.Radiotherapywith or without systemic chemotherapy Fig. 1. T2-weighted image showing brainmagnetic resonance imaging (MRI) with enhancement of the parietooccipital region and corresponding leptomeningeal deposits.