Hypersplenism

Definition

Hypersplenism is a syndrome characterized by splenomegaly and any or all of the following cytopenias: anemia, leukopenia, or thrombocytopenia. Implicit in the definition is that the cytopenias will correct following splenectomy.

The hypersplenism usually is the result of an identifiable pathologic process, but rarely, the cause of the splenomegaly remains elusive, and the hypersplenism is termed primary

Causes of Hypersplenism
 
Hypersplenism can be caused by:
 
Infections  
Bacterial - sepsis, salmonella, abscess
Viral - Hepatitis, CMV, IM
Parasite- Toxoplasma
SABE- Subacute Bacterial Endocarditis,
Malaria
Tuberculosis
 
Inflammation
Felty's syndrome
Systemic lupus erythematosus
Serum sickness
Rheumatic fever
Sarcoidosis
 
Congestive
Cirrhosis
Portal Vein Thrombosis
NCPF- NON Cirrhotic Portal hypertension
Budd Chiari
Splenic vein Thrombosis
 
Haematological
Red cell disorders, Sickle cell, thalassemia, ITP

Malignancy
Myeloproliferative                     Haemangioma
Leukemia                                  Lymphangioma
Lymphoma                                Fibroma
Polycythemia
Histiocytosis
 
Storage
Gaucher
Nieman Picks
 
Misc
Amyloidosis
Cysts

In hypersplenism, the thrombocytopenia is moderately severe (platelet counts of 50 X  109/L to 150 X 109/L). Severe thrombocytopenia (less than 20 ? 109/L) suggests another diagnosis. Therefore, it is unusual for patients with hypersplenism to have evidence of hemostatic impairment attributable to thrombocytopenia, or to need specific interventions to raise the platelet count.

Thrombocytopenia is likely to be caused by hypersplenism when (a) splenomegaly is present, (b) the thrombocytopenia is mild to moderate in severity, (c) moderately reduced neutrophil and low-normal hemoglobin levels are found, and (d) no or minimal evidence for impaired hematopoiesis is observed on bone marrow examination

Treatment

Total or partial splenectomy

Partial splenic embolization

TIPS
Porto systemic shunting

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