SplenomegalyDone by

PROF/ GOUDA ELLABBAN

Splenic anatomy and function

A normal spleen weighs 150 g Normal is 11-13 cm not easily palpable. Spleens that are prominent below the costal margin

typically weigh 750-1000 g. Spleens weighing 400-500 g indicate splenomegaly

Functions The 4 most important normal functions of the

spleen are : (1) clearance of microorganisms and particulate

antigens from the blood stream; (2) synthesis of immunoglobulin G (IgG), (3) removal of abnormal red blood cells (RBCs);

and (4) embryonic hematopoiesis in certain diseases

Causes of enlargement : immune response work hypertrophy such as in subacute

bacterial endocarditis or infectious mononucleosis; RBC destruction work hypertrophy such as in hereditary

spherocytosis or thalassemia major; congestive such as in splenic vein thrombosis or portal

hypertension; myeloproliferative such as in chronic myeloid metaplasia; infiltrative such as in sarcoidosis and some neoplasms; neoplastic such as in chronic lymphocytic leukemia and the

lymphomas. Miscellaneous causes of splenomegaly include.. trauma,

cysts, hemangiomas, metastasis, giant abscess, and certain drugs (eg, RhoGAM).

History The most common history is mild abdominal pain

Febrile illness (infectious) Pallor, dyspnea, bruising, and/or petechiae

(hemolytic process) History of liver disease (congestive) Weight loss, constitutional symptoms (neoplastic) Pancreatitis (splenic vein thrombosis) Alcoholism, hepatitis (cirrhosis)

Physical Spleen size is not a reliable guide to splenic

function, and palpable spleens are not always abnormal

Additional signs that identify possible etiologies of splenomegaly include the following: Signs of cirrhosis (eg, asterixis, jaundice,

telangiectasias, gynecomastia, caput medusa, ascites) Heart murmur (endocarditis, congestive failure) Jaundice Scleral icterus (spherocytosis, cirrhosis) Petechiae (any cause of thrombocytopenia)

Deferential Diagnosis : Berylliosis

Budd-Chiari Syndrome Cirrhosis Hepatitis, Viral Histoplasmosis Hodgkin Disease Immune Thrombocytopenic Purpura Infectious Mononucleosis Infective Endocarditis Iron Deficiency Anemia Leishmaniasis Malaria Myeloproliferative Disease Portal Vein Obstruction Sepsis, Bacterial Sickle Cell Anemia Spherocytosis, Hereditary Splenic Abscess Systemic Lupus Erythematosus Thalassemia, Alpha Thalassemia, Beta Tuberculosis

Symptomatic HIV infection Leukocyte disorders Trypanosomiasis Immune hemolytic anemias Angioimmunoblastic lymphadenopathy Drug reactions with serum sickness syndromes Splenic vein obstruction Congestive heart failure Banti disease Gaucher disease Niemann-Pick disease Mucopolysaccharidosis Hyperlipidemias Lymphomas Histiocytosis X Idiopathic splenomegaly Ovalocytosis

Lab Studies: Perform a CBC count with platelet count. Criteria for a diagnosis of hypersplenism

include the following:

Anemia, leukopenia, thrombocytopenia, or combinations thereof, plus cellular bone marrow, splenomegaly, and improvement after splenectomy

Imaging Studies: CT scan Splenoportography

This is used to evaluate portal vein patency and the distribution of collaterals before shunt operations for cirrhosis.

Angiography: Findings are used to differentiate splenic cysts from other splenic

tumors. Spleen scans

Label erythrocytes with chromium Cr 51, mercury Hg 197, rubidium Rb 81, or technetium Tc 99m, and alter the cells by treatment with heat, antibody, chemicals, or metal ions so that the spleen sequesters them after infusion.

A spleen scan is a good noninvasive technique for evaluating spleen size; a close correlation exists between spleen length on scan images and spleen weight after splenectomy.

A spleen scan is useful for detecting space-occupying lesions in the splenic substance, evaluating loss of spleen functions, assessing for the absence of a spleen, or determining the presence of an accessory spleen.

Ultrasound: Size Histological

Treatment Medical Care: Chemotherapy is used for hematologic malignancy. Antibiotics are used for infection, with the exception

of that associated with splenic abscess. This requires surgical intervention.

Immunosuppression is used for autoimmune or inflammatory disorders, treatment of cirrhosis, and CHF.

All patients scheduled for elective splenectomy (either diagnostic or therapeutic) should receive a pneumococcal vaccine. Also consider administering prophylaxis against Haemophilus influenzae and Neisseria meningitidis.

Surgical Care: Splenectomy: It’s is indicated to help control or stage basic disease. These diseases can include hereditary spherocytosis,

autoimmune thrombocytopenia or hemolysis, or Hodgkin disease (as part of a staging celiotomy.

Splenectomy is also indicated for the treatment of chronic, severe hypersplenism. This can occur in conditions such as hairy cell leukemia, Felty syndrome, agnogenic myeloid metaplasia, thalassemia major, Gaucher disease, hemodialysis splenomegaly, or splenic vein thrombosis.

Splenectomy allows treatment of hematologic disease: Thrombotic thrombocytopenic purpura (TTP

The use of open versus laparoscopic splenectomy (LS) is now a controversy that has yet to be decided.

Complications :

The complication rate for LS remains low, with extremely rare wound or bleeding complications.

Problems inherent to laparoscopic treatment of splenomegaly include structural friability of the spleen, increased difficulties in the manipulation of an enlarged spleen, and exposure and access to the splenic hilum.

Occasionally, severe thrombocytosis occurs following splenectomy, and this requires therapy to reduce the platelet count and prevent thrombotic complications.

This is most common in patients with massive splenomegaly from myeloproliferative disorders.

An onset of fever several days following splenectomy can be due to a recrudescence of malaria.

Outpatient care consists of

2 main focus areas:

(1) monitoring for thrombocytosis and

(2) monitoring for overwhelming postsplenectomy sepsis (OPSS).

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