Hypersplenismus

Front 1

Indikation zur Splenektomie bei hämatologischen Erkrankungen

Back 1

Beeinflussung der Grundkrankheit
• Hereditäre Sphärozytose, Elliptozytose
• Autoimmunhämolytische Anämie
• Idiopathische thrombozytopenische Purpura
• Thrombotisch-thrombozytopenische Purpura (Moschcowitz)
• Haarzellleukämie
• Prolymphozytenleukämie
Beseitigung des Hypersplenismus
• Osteomyelosklerose
• Chronische myeloische Leukämie
Diagnostik
• Explorative Laparotomie bei malignen Lymphomen
• Entfernung eines Milztumors unklarer Ätiologie

Front 2

Major causes of splenomegaly

Back 2

Congestive
Cirrhosis
Heart failure
Thrombosis of portal, hepatic, or splenic veins
Malignancy
Lymphoma, usually indolent variants
Acute and chronic leukemias
Polycythemia vera
Multiple myeloma and its variants
Essential thrombocythemia
Agnogenic myeloid metaplasia
Primary splenic tumors
Metastatic solid tumors
Infection
Viral - hepatitis, infectious mononucleosis, cytomegalovirus
Bacterial - salmonella, brucella, tuberculosis
Parasitic - malaria, schistosomiasis,toxoplasmosis, leishmaniasis
Infective endocarditis
Fungal
Inflammation
Sarcoid
Serum sickness
Systemic lupus erythematosus
Rheumatoid arthritis (Felty syndrome)
Infiltrative, nonmalignant
Gaucher's disease
Niemann-Pick disease
Amyloid
Glycogen storage disease
Langerhans cell histiocytosis
Hemophagocytic lymphohistiocytosis
Rosai-Dorfman disease
Hematologic (hypersplenic) states
Acute and chronic hemolytic anemias, all etiologies
Sickle cell disease (children)
Following use of recombinant human granulocyte colony-stimulating factor

Front 3

OVERVIEW OF SPLENIC FUNCTION

Back 3

• The spleen is a hematopoietic organ, which, at various times during gestation and/or extrauterine life, is capable of supporting elements of the erythroid, myeloid, megakaryocytic, lymphoid, and monocyte-macrophage (ie, reticuloendothelial) systems.
• In certain disease states (eg, beta thalassemia major, primary myelofibrosis), it may become the site of extramedullary hematopoiesis and contain developing erythroid, myeloid, and megakaryocytic precursors.
• the spleen plays a key role in providing an environment for the immunologic response.
• in the absence of the spleen, antibody production may be significantly diminished.
• The spleen acts as a reservoir for platelets;
• one-third of the circulating platelet mass is temporarily sequestered within a normal sized spleen, and up to 90 percent may be found within a markedly enlarged spleen.
• When the spleen is removed, this reservoir function also is removed, and a common occurrence is for the platelet count to temporarily exceed 1 x 106/microL after splenectomy in a normal child, without an increased risk for thrombosis

Front 4

SPLEEN SIZE

Back 4

• The spleen weighs 11 g at birth, 55 gm at six years of age, and 125 g (range 100 to 250 g) at puberty
• The median splenic weight in adults is about 150 grams
• It is not usually palpable, but may be felt in children, adolescents, and some adults, especially those of asthenic build

Front 5

CAUSES OF AN ENLARGED SPLEEN

Back 5

S-P-L-E-E-N:
• Sequestration of red blood cells, as in congenital spherocytosis or other congenital or acquired hemolytic anemias
• Proliferation secondary to chronic inflammation or infection, as in systemic lupus erythematosus, rheumatoid arthritis, and infective endocarditis
• Lipid deposition disorders such as Gaucher or Niemann-Pick disease
• Endowment as in congenital causes for splenomegaly, including splenic hemangioma, hamartoma, or cysts
• Engorgement caused by splenic trauma with intracapsular hematoma formation, sequestration crisis in sickle cell disease, chronic heart failure, or portal hypertension
• iNvasion with granulomatous, histiocytic, lymphoproliferative, or malignant hematologic disease.

Front 6

most common
splenomegaly

Back 6

Children
• an exuberant response of the immune system to infections by many different agents,
• disorders of immune regulation, or
• abnormal destruction of red blood cells.
• However, infiltration with neoplastic or storage disease cells, abnormal splenic blood flow as in portal hypertension, and space-occupying lesions also must be considered.
Adult
• Liver disease — 33 percent (cirrhosis)
• Hematologic malignancy — 27 percent (lymphoma)
• Infection — 23 percent (AIDS, endocarditis)
• Congestion or inflammation — 8 percent (congestive failure)
• Primary splenic disease — 4 percent (splenic vein thrombosis)
• Other or unknown — 5 percent

Front 7

Causes of a massively enlarged spleen

Back 7

• Child
• Leukemia (lymphoid or myeloid, usually chronic, as in chronic myeloid leukemia)
• Thalassemia major
• Lymphoma, usually the more indolent variants
• Langerhans cell histiocytosis
• Autoimmune lymphoproliferative syndrome (Canale Smith syndrome)
• Castleman's disease
• Gaucher disease
• AIDS with Mycobacterium avium complex
• Kala-azar
• Hyperreactive malarial splenomegaly syndrome, also called tropical splenomegaly syndrome
• Hemophagocytic lymphohistiocytosis/familial erythrophagocytic lymphohistiocytosis
• Adult
• Chronic myeloid leukemia
• Myelofibrosis, idiopathic or post-polycythemic
• Gaucher disease
• Lymphoma, usually indolent, including hairy cell leukemia
• Kala-azar (visceral leishmaniasis)
• Hyperreactive malarial splenomegaly syndrome, also called tropical splenomegaly syndrome [84]
• Thalassemia major
• AIDS with Mycobacterium avium complex

Front 8

EVALUATION OF THE CHILD WITH SPLENOMEGALY

Back 8

• accurate history (including recent travel information),
• physical examination,
• complete blood count with white blood cell differential and platelet count,
• liver function studies,
• urinalysis, and
• chest x-ray.
• The complete blood count and examination of the peripheral blood smear should detect evidence of cytopenias (eg, neutropenia, anemia, and/or thrombocytopenia), congenital hemolytic anemias (including sickle cell disease), infection, bone marrow invasion, or malignant disease.
• Testing for the presence of antibodies to Epstein-Barr virus, cytomegalovirus, parvovirus, HIV-1, or reduced acid beta-glucosidase activity in white blood cells in Gaucher disease should be considered when no other causes for splenomegaly are apparent.
• In some cases, CT examination of the chest and abdomen may be considered to evaluate the patient for disseminated or intraabdominal malignancy, such as lymphoma or neuroblastoma, advanced liver disease, or portal hypertension.