Macrophage Activation Syndrome (MAS) is a life-threatening hyperinflammatory disorder characterized by excessive activation of macrophages and T lymphocytes. It is considered a severe complication of systemic autoimmune diseases, particularly systemic juvenile idiopathic arthritis (sJIA) and systemic lupus erythematosus (SLE). MAS belongs to the spectrum of hemophagocytic lymphohistiocytosis (HLH) and is associated with cytokine storms, leading
Lymphoid blast phase Philadelphia chromosome-positive chronic myelocytic leukemia (CML) represents an advanced stage of CML characterized by the accumulation of immature lymphoid blast cells in the bone marrow and blood. This transition from chronic phase to blast phase significantly worsens prognosis and requires immediate therapeutic intervention. Pathophysiology of Lymphoid Blast Phase CML CML is driven
Lysosomal alpha-1,4-glucosidase deficiency, also known as Pompe disease or Glycogen Storage Disease Type II (GSD II), is a rare genetic disorder caused by a deficiency of the enzyme acid alpha-glucosidase (GAA). This enzyme is responsible for breaking down glycogen into glucose within lysosomes. Without sufficient GAA, glycogen accumulates in muscle tissues, leading to progressive muscle
Lysosomal Acid Lipase Deficiency (LAL-D) is a rare genetic disorder affecting the body’s ability to break down lipids. The condition results from mutations in the LIPA gene, which encodes the enzyme lysosomal acid lipase (LAL). This enzyme is crucial for breaking down cholesterol esters and triglycerides within lysosomes. Causes and Genetic Basis of LAL-D LAL-D
Lymphomatous meningitis (LM), also known as leptomeningeal lymphoma, is a rare but severe complication of systemic or primary central nervous system (CNS) lymphoma. It occurs when malignant lymphocytes infiltrate the meninges, leading to neurological dysfunction. Early recognition and treatment are critical to improving patient outcomes. Causes and Pathophysiology LM results from the hematogenous or direct
Lymphogranuloma venereum (LGV) is a sexually transmitted infection (STI) caused by specific serovars (L1, L2, L3) of Chlamydia trachomatis. It primarily affects the lymphatic system and is more prevalent among men who have sex with men (MSM). Early detection and treatment are crucial to preventing severe complications. Causes and Transmission LGV is caused by Chlamydia
Lymphocutaneous sporotrichosis is a subcutaneous fungal infection caused by Sporothrix schenckii complex species. This infection primarily affects the skin and lymphatic system, often following minor trauma involving plant material. It is commonly seen in gardeners, florists, and individuals handling organic matter. Causes and Transmission The primary cause of lymphocutaneous sporotrichosis is infection with Sporothrix schenckii,
Lymphoblastic lymphoma (LBL) is a rare and aggressive subtype of non-Hodgkin’s lymphoma (NHL), primarily affecting children, adolescents, and young adults. It originates from immature lymphocytes (lymphoblasts) and is closely related to acute lymphoblastic leukemia (ALL). LBL is classified into: Early diagnosis and intensive chemotherapy are crucial for improving survival rates. Pathophysiology of Lymphoblastic Lymphoma LBL
Lymphangioleiomyomatosis (LAM) is a rare, progressive lung disease primarily affecting women of childbearing age. It is characterized by the abnormal proliferation of smooth muscle-like cells (LAM cells) in the lungs, leading to cystic destruction, airflow obstruction, and eventual respiratory failure. LAM can occur sporadically or in association with Tuberous Sclerosis Complex (TSC-LAM). Pathophysiology of LAM
Lymph node-positive colorectal carcinoma (CRC) occurs when cancer cells spread from the primary tumor in the colon or rectum to nearby lymph nodes. This stage signifies a more advanced disease and influences treatment decisions, prognosis, and recurrence risk. Detecting and managing lymph node involvement is crucial to improving patient survival rates. Pathophysiology of Lymph Node