HyperspleenismDefinisi: merupakan kondisi overaktif dari spleemPenyebab:myeloprolifeferative disorder, lymphoproliferative, disorder, storage disorder, connective tissue disorder, infection disease.Tanda cardinal: splenomegaly, cytopenia, rasa cepat kenyang setelah makan, nyeri perut LUQCommon Causes of Splenomegaly*

Type ExamplesCongestive Cirrhosis

External compression or thrombosis of portal or splenic veinsCertain malformations of the portal venous vasculature

Infectious and inflammatory

Acute infections (eg, infectious mononucleosis, infectious hepatitis, subacute bacterial endocarditis, psittacosis)Chronic infections (eg, miliary TB, malaria, brucellosis, kala-azar, syphilis)SarcoidosisSecondary amyloidosisConnective tissue disorder (eg, SLE, Felty's syndrome)

Myeloproliferative and lymphoproliferative

Myelofibrosis with myeloid metaplasiaLymphomasLeukemias, especially chronic lymphocytic and chronic myelocyticPolycythemia veraPrimary thrombocythemia

Chronic hemolytic† RBC shape abnormalities (eg, hereditary spherocytosis, hereditary elliptocytosis)Hemoglobinopathies, including thalassemias, sickle cell hemoglobin variants (eg, hemoglobin S-C disease), and congenital Heinz body hemolytic anemiasRBC enzymopathies (eg, pyruvate kinase deficiency)

Storage diseases Lipoid (eg, Gaucher's, Niemann-Pick, Hand-Schüller-Christian, and Wolman's diseases)Nonlipoid (eg, Letterer-Siwe disease)

Structural Splenic cysts, usually caused by resolution of previous intrasplenic hematoma

Splenomegaly: ukuran spleen > 12 cm, klasifikasi: 1. Moderate splenomegaly: uk 11 – 20 cm, severe splenomegaly uk >20 cm Symptom: nyeri perut, nyeri dada, anemia.Sign: massa abdomen di LUQ, castell’s sign, traube’s space, USGTherapy: splenektomiIndikasi splenektomiIt is removed under the following circumstances:

1. When it becomes very large such that it becomes destructive to platelets/red cells2. For diagnosing certain lymphomas3. Certain cases of wandering spleen4. When platelets are destroyed in the spleen as a result of an auto-immune process (see also idiopathic thrombocytopenic purpura)5. When the spleen bleeds following physical trauma6. Following spontaneous rupture7. For long-term treatment of congenital erythropoietic porphyria (CEP) if severe hemolytic anemia develops[1]

8. The spread of gastric cancer to splenic tissue9. When using the splenic artery for kidney revascularisation in renovascular hypertension.

Workup : setelah splenektomi pasien harus diberi vaksin Haemophilus influenzae, Streptococcus pneumoniae, Meningococcus dan influenza vaccinations. Pemberian antibiotic profilaksis juga wajib diberikan pada beberapa kasus.

Indications for Splenectomy or Radiation Therapy in

HypersplenismIndication ExamplesHemolytic syndromes in which splenomegaly further shortens the survival of intrinsically abnormal RBCs

Hereditary spherocytosisThalassemia

Severe pancytopenia associated with massive splenomegaly

Lipid-storage diseases*

Vascular insults affecting the spleen

Recurrent infarctionsBleeding esophageal varices associated with excessive splenic venous return

Mechanical encroachment by other abdominal organs

Stomach with early satietyCalyceal obstruction in left kidney

Excessive bleeding Hypersplenic thrombocytopenia

*The spleen may be up to 30 times larger than normal.

HyperspleenismDefinisi: merupakan kondisi overaktif dari spleemPenyebab:myeloprolifeferative disorder, lymphoproliferative, disorder, storage disorder, connective tissue disorder, infection disease.Tanda cardinal: splenomegaly, cytopenia, rasa cepat kenyang setelah makan, nyeri perut LUQ

Common Causes of Splenomegaly*

Type Examples

Congestive CirrhosisExternal compression or thrombosis of portal or splenic veinsCertain malformations of the portal venous vasculature

Infectious and inflammatory Acute infections (eg, infectious mononucleosis, infectious hepatitis, subacute bacterial endocarditis, psittacosis)Chronic infections (eg, miliary TB, malaria, brucellosis, kala-azar, syphilis)SarcoidosisSecondary amyloidosisConnective tissue disorder (eg, SLE, Felty's syndrome)

Myeloproliferative and lymphoproliferative

Myelofibrosis with myeloid metaplasiaLymphomasLeukemias, especially chronic lymphocytic and chronic myelocyticPolycythemia veraPrimary thrombocythemia

Chronic hemolytic† RBC shape abnormalities (eg, hereditary spherocytosis, hereditary elliptocytosis)Hemoglobinopathies, including thalassemias, sickle cell hemoglobin variants (eg, hemoglobin S-C disease), and congenital Heinz body hemolytic anemiasRBC enzymopathies (eg, pyruvate kinase deficiency)

Storage diseases Lipoid (eg, Gaucher's, Niemann-Pick, Hand-Schüller-Christian, and Wolman's diseases)Nonlipoid (eg, Letterer-Siwe disease)

Structural Splenic cysts, usually caused by resolution of previous intrasplenic hematoma

Splenomegaly: ukuran spleen > 12 cm, klasifikasi: 1. Moderate splenomegaly: uk 11 – 20 cm, severe splenomegaly uk >20 cm Symptom: nyeri perut, nyeri dada, anemia.Sign: massa abdomen di LUQ, castell’s sign, traube’s space, USGTherapy: splenektomiIndikasi splenektomiIt is removed under the following circumstances:

10. When it becomes very large such that it becomes destructive to platelets/red cells11. For diagnosing certain lymphomas12. Certain cases of wandering spleen13. When platelets are destroyed in the spleen as a result of an auto-immune process (see also

idiopathic thrombocytopenic purpura)14. When the spleen bleeds following physical trauma15. Following spontaneous rupture16. For long-term treatment of congenital erythropoietic porphyria (CEP) if severe hemolytic anemia

develops[1]

17. The spread of gastric cancer to splenic tissue18. When using the splenic artery for kidney revascularisation in renovascular hypertension.

Workup : setelah splenektomi pasien harus diberi vaksin Haemophilus influenzae, Streptococcus pneumoniae, Meningococcus dan influenza vaccinations. Pemberian antibiotic profilaksis juga wajib diberikan pada beberapa kasus.

Indications for Splenectomy or Radiation Therapy in Hypersplenism

Indication Examples

Hemolytic syndromes in which splenomegaly further shortens the survival of intrinsically abnormal RBCs

Hereditary spherocytosisThalassemia

Severe pancytopenia associated with massive splenomegaly

Lipid-storage diseases*

Vascular insults affecting the spleen Recurrent infarctionsBleeding esophageal varices associated with excessive splenic venous return

Mechanical encroachment by other abdominal organs

Stomach with early satietyCalyceal obstruction in left kidney

Excessive bleeding Hypersplenic thrombocytopenia

*The spleen may be up to 30 times larger than normal.

HyperspleenismDefinisi: merupakan kondisi overaktif dari spleemPenyebab:myeloprolifeferative disorder, lymphoproliferative, disorder, storage disorder, connective tissue disorder, infection disease.Tanda cardinal: splenomegaly, cytopenia, rasa cepat kenyang setelah makan, nyeri perut LUQ

Common Causes of Splenomegaly*

Type Examples

Congestive CirrhosisExternal compression or thrombosis of portal or splenic veinsCertain malformations of the portal venous vasculature

Infectious and inflammatory Acute infections (eg, infectious mononucleosis, infectious hepatitis, subacute bacterial endocarditis, psittacosis)Chronic infections (eg, miliary TB, malaria, brucellosis, kala-azar, syphilis)SarcoidosisSecondary amyloidosisConnective tissue disorder (eg, SLE, Felty's syndrome)

Myeloproliferative and lymphoproliferative

Myelofibrosis with myeloid metaplasiaLymphomasLeukemias, especially chronic lymphocytic and chronic myelocyticPolycythemia veraPrimary thrombocythemia

Chronic hemolytic† RBC shape abnormalities (eg, hereditary spherocytosis, hereditary elliptocytosis)Hemoglobinopathies, including thalassemias, sickle cell hemoglobin variants (eg, hemoglobin S-C disease), and congenital Heinz body hemolytic anemiasRBC enzymopathies (eg, pyruvate kinase deficiency)

Storage diseases Lipoid (eg, Gaucher's, Niemann-Pick, Hand-Schüller-Christian, and Wolman's diseases)Nonlipoid (eg, Letterer-Siwe disease)

Structural Splenic cysts, usually caused by resolution of previous intrasplenic hematoma

Splenomegaly: ukuran spleen > 12 cm, klasifikasi: 1. Moderate splenomegaly: uk 11 – 20 cm, severe splenomegaly uk >20 cm Symptom: nyeri perut, nyeri dada, anemia.Sign: massa abdomen di LUQ, castell’s sign, traube’s space, USGTherapy: splenektomiIndikasi splenektomiIt is removed under the following circumstances:

1. When it becomes very large such that it becomes destructive to platelets/red cells2. For diagnosing certain lymphomas3. Certain cases of wandering spleen4. When platelets are destroyed in the spleen as a result of an auto-immune process (see also

idiopathic thrombocytopenic purpura)5. When the spleen bleeds following physical trauma6. Following spontaneous rupture7. For long-term treatment of congenital erythropoietic porphyria (CEP) if severe hemolytic anemia

develops[1]

8. The spread of gastric cancer to splenic tissue9. When using the splenic artery for kidney revascularisation in renovascular hypertension.

Workup : setelah splenektomi pasien harus diberi vaksin Haemophilus influenzae, Streptococcus pneumoniae, Meningococcus dan influenza vaccinations. Pemberian antibiotic profilaksis juga wajib diberikan pada beberapa kasus.

Indications for Splenectomy or Radiation Therapy in Hypersplenism

Indication Examples

Hemolytic syndromes in which splenomegaly further shortens the survival of intrinsically abnormal RBCs

Hereditary spherocytosisThalassemia

Severe pancytopenia associated with massive splenomegaly

Lipid-storage diseases*

Vascular insults affecting the spleen Recurrent infarctionsBleeding esophageal varices associatedjg with excessive splenic venous return

Mechanical encroachment by other abdominal organs

Stomach with early satietyCalyceal obstruction in left kidney

Excessive bleeding Hypersplenic thrombocytopenia

*The spleen may be up to 30 times larger than normal.