Abstract
Background: Enlarged lymph nodes are a common complaint in a Pediatrician’s office. Diagnosis of reactive lymphadenopathy secondary to infectious, inflammatory, immune dysregulation calls for clinical investigation, including a thorough history, physical exam, imaging, and less often, a biopsy of the lymph node. Here we discuss a rare presentation of extensive generalized, chronic, waxing, and waning lymphadenopathy diagnosed as Progressive Transformation of Germinal Centers (PTGC) and the course of illness over eight years follow up period. Discussion: Progressive Transformation of Germinal Centers (PTGC) is considered a benign condition, but extensive recurrent generalized lymphadenopathy in a very young child has not been reported before. This case demonstrates the importance of long-term follow-up and tailoring the diagnostic work-up and management based on new signs and symptoms. Here we focus on the clinical considerations and management of complex presentation of a common clinical finding.
Highlights
Clinical presentation: A two-year-old caucasian male presented to the emergency department with a one-month history of a lump in the right axilla
Observation for 3–4 weeks is a reasonable approach in a young child with isolated lymph node enlargement without any signs and symptoms suggestive of severe systemic illness or malignancy [7,9]
As demonstrated in this case and similar reports, Progressive Transformation of Germinal Centers (PTGC) presents with varied clinical presentation and poses diagnostic and management challenges
Summary
Evaluation by rheumatologist showed normal Anti-Neutrophil Cytoplasmic Antibody (ANCA), angiotensin converting enzyme (ACE), lysozyme, anti–Sjögren’s-syndrome-related antigen A (SSA) autoantibodies, called anti-Ro and anti-SSB, rheumatoid factor, and total Creatine Phosphokinase (CPK) level were normal, suggesting against autoimmune or inflammatory disorder. The IgG4-positive multiorgan lymphoproliferative syndrome is a distinct clinical entity characterized by elevated serum IgG level and increased IgG4 + plasma cell infiltration in involved tissues (pancreas, lacrimal and salivary glands, and lymph nodes) [1,2]. Our patient did not have systemic symptoms except for mild fatigue Around age eight, he presented with recurrent and worsening abdominal pain. The child continues to be thriving with age-appropriate growth and development He continues to have multiple palpable pathologically enlarged lymph nodes. We continue periodic physical exams (every six months) to determine the need for further imaging such as ultrasonography and frequent discussion with family about overall clinical status, which has helped assess the need for imaging and or biopsy of the lymph node
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