storage diseases


splenomegaly

Enlarged spleen Enlargement of spleen for any reason, which is usually a manifestation of underlying disease; the only specific finding in splenomegaly is dragging sensation in the upper right quadrant; megalic spleens may reach 4.0+ kg–eg, in agnogenic myeloid metaplasia Splenomegaly Congestion Cirrhosis, CHF, thrombosis of portal or splenic veins Infection • Bacteria Brucellosis, infective carditis agents, syphilis, TB, typhoid fever • Fungi Histoplasmosis • Parasites Echinococcosis, leishmaniasis, malaria, schistosomiasis, toxoplasmosis, trypanosomiasis • Viruses CMV, EBV Inflammatory/immune-related Rheumatoid arthritis, SLE Hematopoietic disease/Lymphoid function • Malignant Leukemias, eg ALL, CLL, myeloproliferative disorders–eg agnogenic myeloid metaplasia, CML, multiple myeloma, polycythemia vera; lymphomas–Hodgkin's disease, NHL • Nonmalignant Hemolytic anemia, histiocytosis, ITP Storage diseases Gaucher's disease, mucopolysaccharidosis, Niemann-Pick disease Etc Amyloidosis, cysts, hypersplenism, metastases, primary tumors

storage diseases

A range of metabolic disorders in which various substances accumulate in abnormal amounts in certain body tissues or organs such as the liver. See also SPHINGOLIPIDOSES.