Chapter 060. Enlargement of Lymph Nodes and Spleen (Part 7)

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Differential Diagnosis Many of the diseases associated with splenomegaly are listed in Table 60-2. They are grouped according to the presumed basic mechanisms responsible for organ enlargement: 1. Hyperplasia or hypertrophy related to a particular splenic function such as reticuloendothelial hyperplasia (work hypertrophy) in diseases such as hereditary spherocytosis or thalassemia syndromes that require removal of large numbers of defective red blood cells; immune hyperplasia in response to systemic infection (infectious mononucleosis, subacute bacterial endocarditis) or to immunologic diseases (immune thrombocytopenia, SLE, Felty's syndrome). 2. Passive congestion due to decreased blood flow from the spleen in conditions that produce portal hypertension (cirrhosis, BuddChiari syndrome, congestive heart...

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Chapter 060. Enlargement of Lymph Nodes and Spleen (Part 7) Differential Diagnosis Many of the diseases associated with splenomegaly are listed in Table 60-2. They are grouped according to the presumed basic mechanisms responsible for organ enlargement: 1. Hyperplasia or hypertrophy related to a particular splenic function such as reticuloendothelial hyperplasia (work hypertrophy) in diseases such as hereditary spherocytosis or thalassemia syndromes that require removal of large numbers of defective red blood cells; immune hyperplasia in response to systemic infection (infectious mononucleosis, subacute bacterial endocarditis) or to immunologic diseases (immune thrombocytopenia, SLE, Felty's syndrome).
2. Passive congestion due to decreased blood flow from the spleen in conditions that produce portal hypertension (cirrhosis, Budd- Chiari syndrome, congestive heart failure). 3. Infiltrative diseases of the spleen (lymphomas, metastatic cancer, amyloidosis, Gaucher's disease, myeloproliferative disorders with extramedullary hematopoiesis). Table 60-2 Diseases Associated with Splenomegaly Grouped by Pathogenic Mechanism Enlargement Due to Enlargement Due to Abnormal Increased Demand for Splenic Splenic or Portal Blood Flow Function Reticuloendothelial system Cirrhosis hyperplasia (for removal of defective Hepatic vein obstruction erythrocytes) Portal vein obstruction, intrahepatic Spherocytosis or extrahepatic Early sickle cell anemia Cavernous transformation of the
Ovalocytosis portal vein Thalassemia major Splenic vein obstruction Hemoglobinopathies Splenic artery aneurysm Paroxysmal nocturnal Hepatic schistosomiasis hemoglobinuria Congestive heart failure Pernicious anemia Hepatic echinococcosis Immune hyperplasia Portal hypertension (any cause Response to infection (viral, including the above): "Banti's disease" bacterial, fungal, parasitic) Infiltration of the Spleen Infectious mononucleosis AIDS Intracellular or extracellular depositions Viral hepatitis Amyloidosis Cytomegalovirus Gaucher's disease Subacute bacterial endocarditis Niemann-Pick disease
Bacterial septicemia Tangier disease Congenital syphilis Hurler's syndrome and other mucopolysaccharidoses Splenic abscess Hyperlipidemias Tuberculosis Benign and malignant cellular Histoplasmosis infiltrations Malaria Leukemias (acute, chronic, Leishmaniasis lymphoid, myeloid, monocytic) Trypanosomiasis Lymphomas Ehrlichiosis Hodgkin's disease Disordered immunoregulation Myeloproliferative syndromes (e.g., polycythemia vera, essential Rheumatoid arthritis (Felty's thrombocytosis) syndrome) Angiosarcomas Systemic lupus erythematosus Metastatic tumors (melanoma is most common) Collagen vascular diseases
Serum sickness Eosinophilic granuloma Immune hemolytic anemias Histiocytosis X Immune thrombocytopenias Hamartomas Immune neutropenias Hemangiomas, fibromas, lymphangiomas Drug reactions Splenic cysts Angioimmunoblastic lymphadenopathy Unknown Etiology Sarcoidosis Idiopathic splenomegaly Thyrotoxicosis (benign lymphoid hypertrophy) Berylliosis Interleukin 2 therapy Iron-deficiency anemia Extramedullary hematopoiesis Myelofibrosis Marrow damage by toxins, radiation, strontium
Marrow infiltration by tumors, leukemias, Gaucher's disease