Abstract
Lymphomatoid granulomatosis (LYG) is an extremely rare angio-centric and angio-destructive B-cell lymphoproliferative disease. Driven by Epstein-Barr virus (EBV), LYG predominantly involves the bilateral lungs. Commonly presenting as multiple nodules in the lung, pulmonary LYG can masquerade as various infectious diseases, vasculitis, lung cancer, or other metastatic neoplasm. It is difficult to be diagnosed and is always neglected by clinicians. No standardized therapeutic regimens for LYG has been established yet now. Hemophagocytic lymphohistiocytosis (HLH), a life-threatening condition caused by abnormal activation of macrophages and T-cells, is characterized by fever, hepatosplenomegaly, pancytopenia, hypercytokinemia, and the presence of hemophagocytosis within the bone marrow, liver, spleen, or other lymphatic tissue. We herein report a 55-year-old man with recurrent fever, severe jaundice, and multiple high-density opacities and nodules in both lungs, who was finally diagnosed with pulmonary LYG (Grade 3) manifested with secondary HLH. Administration of HLH-1994 protocol led to the rapid control of the symptoms caused by HLH. Rituximab-based combination therapy was useful yet LYG (Grade 3) progressed rapidly. This case demonstrates that tissue biopsy is essential for early pathological diagnosis and effective treatment of LYG.
Highlights
Lymphomatoid granulomatosis (LYG), an extremely rare B-cell lymphoproliferative disease, predominantly involves the bilateral lungs but can affect skin, central nervous system, kidney, liver, spleen, and other organs
Diminished or absent activity of nature killer (NK) cells and CTLs leads to a decrease in granule release, triggering the uncontrolled stimulation of macrophages, sustained inflammatory response and cytokine storm
Transplantation-induced Hemophagocytic lymphohistiocytosis (HLH) or post-HSCT HLH is a specific entity of secondary HLH and infections, especially Epstein-Barr virus (EBV) or cytomegalovirus infections, are the potential trigger of post-HSCT HLH, the risk of which could be reduced by etoposide-based conditioning regimen [25]
Summary
Lymphomatoid granulomatosis (LYG), an extremely rare B-cell lymphoproliferative disease, predominantly involves the bilateral lungs but can affect skin, central nervous system, kidney, liver, spleen, and other organs. Based upon the patient’s clinical presentation and laboratory tests, the diagnosis of secondary HLH was made after fulfilling six out of eight diagnostic criteria for HLH-2004 [10], including fever, splenomegaly, bilineage cytopenia, elevated triglyceride (>3 mmol/L), and/or hypofibrinogenemia (
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