splenomegaly

Splenomegaly refers to enlargement of the spleen. The upper limit of normal adult splenic length is traditionally cited at 12 cm, but lengths upwards of 14 cm can be seen in normal, taller males .

Massive splenomegaly is variably defined, including when the spleen is 5 standard deviations above the mean normal volume (about 943 cm) , heavier than 1000 g  or 1500 g ,longer than 18 cm , or extending into the pelvis or across midline .

Pathology

The causes of splenomegaly are protean, and can be thought of under a number of headings :

• hematological disease
• hemodynamic
• infectious
• storage diseases/metabolic/infiltrative disorders
• neoplastic (non-hematologic)
• traumatic
• connective tissue disorders

Hematological disease

• anemias
  • thalassemia (including beta thalassemia major*)
  • sickle cell disease with splenic sequestration (in young patients prior to developing autosplenectomy)
  • hereditary spherocytosis
  • pyruvate kinase deficiency
  • thrombotic thrombocytopenic purpura (TTP)
  • Plummer-Vinson syndrome
• neoplastic/proliferative/redistribution of hematopoiesis
  • myeloproliferative neoplasms*
    • chronic myeloid leukemia
    • primary myelofibrosis
    • polycythemia vera
    • essential thrombocytosis
    • chronic myelomonocytic leukemia
  • acute leukemia*
    • acute myeloid leukemia
    • acute lymphoblastic leukemia
  • lymphoma / chronic lymphoid neoplasms*
    • chronic lymphocytic leukemia
    • Hodgkin lymphoma
    • non-Hodgkin lymphoma
    • hairy cell leukemia
    • Waldenström macroglobulinemia
  • extramedullary hematopoiesis
  • osteopetrosis
  • idiopathic hypereosinophilic syndrome

Hemodynamic

• cirrhosis: portal hypertension (common)
• congestive splenomegaly (Banti syndrome)
• splenic vein obstruction
• portal vein obstruction
• right heart failure
• cystic fibrosis

Infection

• viral
  • EBV (infectious mononucleosis) (common)
  • AIDS with Mycobacterial avium complex infection*
  • CMV
  • herpes simplex virus
  • rubella
• bacterial
  • tuberculosis (miliary)
  • tularemia
  • subacute bacterial endocarditis
  • typhoid fever
  • brucellosis
  • syphilis
  • abscess
• fungal
  • histoplasmosis (common)
  • candidiasis
• parasitic disease
  • malaria* (hyperreactive malarial splenomegaly syndrome or tropical splenomegaly syndrome)
  • schistosomiasis
  • hydatid disease
  • leishmaniasis (kala-azar)*
• rickettsial
  • typhus

Storage diseases/metabolic/infiltrative disorders

• Gaucher disease*
• glycogen storage disease
• mucopolysaccharidoses
• Niemann-Pick disease
• hemochromatosis
• amyloidosis
• porphyria
• sarcoidosis

Neoplastic (non-hematologic)

• metastases (breast, lung, colon, ovary, melanoma)
• hemangioma
• lymphangioma
• angiosarcoma

Trauma

• hematoma
• pseudocyst

Connective tissue disorders

• rheumatoid arthritis
  • Felty syndrome
  • juvenile rheumatoid arthritis (JRA)
  • Still disease
• systemic lupus erythematosus (SLE)

* may cause massive splenomegaly

Radiographic features

The shape and orientation of a spleen make accurate linear measurement difficult.

On CT, a splenic width measurement (largest anterior-posterior measurement on axial images) of greater than 10.5 cm is the most accurate single measurement for mild to moderate splenomegaly in patients with cirrhosis; while a craniocaudal measurement of greater than 14.6 cm is the most accurate single measurement for massive splenomegaly .

On sonographic assessment, a length of 12 cm is generally considered the upper limit of normal.

See also

• spleen size (pediatric)
• hepatosplenomegaly
• fetal splenomegaly
• fetal hepatosplenomegaly

Siehe auch:

• Tuberkulose
• Leberzirrhose
• Sarkoidose
• Hämangiom
• Osteopetrose
• portale Hypertension
• systemischer Lupus Erythematodes
• Sichelzellenanämie
• Amyloidose
• extramedulläre Hämatopoese
• Osteomyelofibrose
• AIDS
• Felty syndrome
• Morbus Gaucher
• Echinokokkose
• Angiosarkom
• Thalassämie
• Mukopolysaccharidose
• Hämochromatose
• idiopathic hypereosinophilic syndrome
• fetal splenomegaly
• Glykogenspeicherkrankheit
• Rechtsherzinsuffizienz
• pyruvate kinase deficiency
• polycythaemia rubra vera
• congestive splenomegaly
• Hereditäre Sphärozytose
• Waldenström's macroglobulinaemia
• Striktur Pfortader
• Thrombotisch-thrombozytopenische Purpura
• lymphatische Malformationen
• Mononukleose
• splenic involvement in non-Hodgkin lymphoma
• Obstruktion der Milzvene

und weiter:

• fokale Milzläsionen
• Raumforderungen der Milz
• Rheumatoide Arthritis
• hepatosplenomegaly
• Milzinfarkt
• Wolman disease
• Primär biliäre Zirrhose
• chronic lymphocytic leukaemia
• abdominal manifestations of sickle cell disease
• Rai staging system for chronic lymphoid leukaemia
• spontane Milzruptur
• perisplenitis
• Filgrastim
• Splenomegalie Sarkoidose
• primary familial xanthomatosis
• Budd-Chiari-Syndrom
• Littoralzellangiom der Milz
• connatale Zytomegalie
• mononucleose spleen
• Chronische lymphatische Leukämie