Marginal Zone Lymphoma Manifesting as Macrophage Activation | Figure 1
Marginal Zone Lymphoma Manifesting as Macrophage Activation Syndrome: A Case Report
Macrophage activation syndrome (MAS) is a form of secondary hemophagocytic lymphohistiocytosis (HLH) when it occurs in the context of rheumatologic disorders. HLH is a rare and potentially life-threatening syndrome characterized by excessive immune system activation. It is mainly seen in children and can be genetic based or related to infections, malignancies, rheumatologic disorders, or immunodeficiency syndromes. MAS can present with nonspecific symptoms, leading to a delay in diagnosis. This report describes a case of a 64-year-old female with marginal zone lymphoma and systemic lupus erythematosus who presented with a purpuric rash and acute kidney injury. She underwent a kidney biopsy and was diagnosed with MAS. This case highlights the importance of promptly recognizing MAS’s symptoms and signs, allowing timely diagnosis and early therapeutic intervention. This potentially fatal condition tends to respond well to rapid treatment initiation with corticosteroids and to address the underlying condition.
Image description: Petechial rash, purpura, and blisters on patient’s left lower extremity-leukocytoclastic vasculitis.
Similar cases
Mixed connective tissue disease (MCTD) is a rheumatologic overlap syndrome that can present with symptoms of systemic lupus erythematous, scleroderma, and polymyositis. A severe but rare complication that can occur in MCTD is scleroderma renal crisis. This case reports a rare case of scleroderma renal crisis in a patient with MCTD who we treated with plasma exchange for clinical suspicion for an underlying thrombotic thrombocytopenia and mycophenolate mofetil for MCTD. The patient had multiple poor prognostic indicators yet made a full renal recovery in less than 3 months.
Image description: Light microscopy of kidney section stained with hematoxylin-eosin.
Immunoglobulin G4-related disease (IgG4-RD) is a chronic fibrosing inflammatory systemic disorder that has been recognized relatively recently in the medical literature. This case reports the situation of a 66-year-old male with progressive kidney disease diagnosed with tubulointerstitial nephritis due to IgG4-RD.
Chronic lymphocytic leukemia (CLL) is an indolent malignancy characterized by the accumulation of dysfunctional B-cell lymphocytes. In this case, a 65-year-old woman presented with symptoms consistent with HLH potentially connected to undiagnosed CLL.
Further research is needed to explore the relationship between inflammation and the pathogenesis of CLL.
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