SPLENOMEGALY

DEFINATION
Enlargement of spleen beyond it’s normal size is known as Spleenomegaly.

What is an enlarged spleen?

The spleen is an organ located in the upper left quadrant of the abdomen just below the diaphragm and protected under the lower left ribs.

The spleen has a couple of important functions involving blood cells within the body.

1. It filters blood and removes old and damaged red blood cells, bacteria, and other particles as they pass through the maze of blood vessels within the spleen.
2. It produces lymphocytes, a type of white blood cell that produces antibodies and assists immune system.

The filtration system is part of the red pulp while the white pulp of the spleen contains the immune functioning cells

Normally, the spleen is a small organ about the size of a small fist or orange. Splenomegaly describes the situation where the spleen enlarges in size (spleen+megaly=enlargement).

SYMPTOMS
An enlarged spleen ofte causes no symptoms however some patients may have symptoms like

• Weakness
• Poor appetite
• Belching
• Retention of urine
• Constipation
• Excessive thirst
• Feeling of great heat
• Loss of appetite
• Fainting
• Respiratory distress
• Mild fever
• Loss of weight
• Tastelessness
• Pain in abdomen

Tests and diagnosis
An enlarged spleen is usually detected during a physical exam. The doctor can often feel the enlargement by gently examining your left upper abdomen, just under the rib cage.

The diagnosis may be confirmed with blood tests, an X-ray or other imaging studies of the abdomen. For example, an ultrasound or computerized tomography (CT) scan can help the doctor determine the size of your spleen and whether it’s crowding other organs. Magnetic resonance imagining (MRI) can be used to trace blood flow through the spleen.

Depending on the circumstances, the doctor may recommend various blood tests or other diagnostic tests to identify what’s causing the enlarged spleen.

TREATMENT OF SPLEENOMEGALY
After proper sanehan savedan and virechan karma niruhan vasti and anuvasan vasti should be given.

Commonly used drugs
o Piplayadi choornao
o Vangeshvar ras ( Rastantarsar)
o Vidangadikshar
o Rohitakadiyog

Causes

Splenomegaly grouped on the basis of the pathogenic mechanism

Increased function	Abnormal blood flow	Infiltration
Removal of defective RBCs spherocytosis thalassemia hemoglobinopathies nutritional anemias early sickle cell anemia Immune hyperplasia Response to infection (viral,bacterial,fungal,parasitic) mononucleosis, AIDS, viral hepatitis subacute bacterial endocarditis, bacterial septicemia splenic abscess, typhoid fever brucellosis, leptospirosis, tuberculosis histoplasmosis malaria, leishmaniasis, trypanosomiasis ehrlichiosis Disordered immunoregulation rheumatoid arthritis SLE Serum sickness Autoimmune hemolytic anemia Immune thrombocytopenia sarcoidosis drug reactions Extramedullary hematopoiesis Myelofibrosis Marrow infiltration by tumors, leukemias marrow damage by radiation, toxins	Organ Failure cirrhosis congestive heart failure Vascular hepatic vein obstruction portal vein obstruction Budd-Chiari syndrome splenic vein obstruction Infections hepatic schistosomiasis hepatic echinococcosis	Metabolic diseases Gauchers disease Niemann-Pick disease Hurler syndrome and other Mucopolysaccharidoses Amyloidosis Tangier disease Benign and malignant infiltrations Leukemias(acute,chronic,lymphoid and myeloid) lymphomas(Hodgkins and non-hodgkins) myeloproliferative disorders metastatic tumors(commonly melanoma) histiocytosis X Hemangioma,lymphangioma splenic cysts hamartomas eosinophilic granuloma

 

The causes of massive splenomegaly (>1000 g) are much fewer and include:

• thalassemia
• visceral leishmaniasis (Kala Azar)
• schistosomiasis
• chronic myelogenous leukemia
• chronic lymphocytic leukemia
• lymphomas
• hairy cell leukemia
• myelofibrosis
• polycythemia vera
• Gauchers disease
• sarcoidosis
• autoimmune hemolytic anemia
• malaria

Home Care

Appropriate limitation of activity, including avoiding contact sports, will help prevent trauma that might cause the spleen to rupture.

Care will be required for the specific condition causing the splenomegaly. Follow the instructions given by your health care provider regarding appropriate care.

Treatment

If the splenomegaly underlies hypersplenism, a splenectomy is indicated and will correct the hypersplenism. However, the underlying cause of the hypersplenism will most likely remain, so a thorough diagnostic workup is still indicated, as leukemia, lymphoma, and other serious disorders can cause hypersplenism and splenomegaly. After splenectomy, however, patients have an increased risk for infectious diseases.

After splenectomy, patients should be vaccinated against Haemophilus influenzae, Streptococcus pneumoniae, and Meningococcus. They should also receive annual influenza vaccinations. Long-term prophylactic antibiotics may be given in certain cases.

Complications

• Postsplenectomy infection
• Fulminant, life-threatening infection represents a major long-term sequela after splenectomy in patients with splenomegaly. Splenic macrophages play a major role in filtering and phagocytizing bacteria and parasitized blood cells from the circulation. In addition, the spleen is a significant source of antibody production.
• Overwhelming postsplenectomy infection (OPSI), also known as postsplenectomy sepsis syndrome, begins as a nonspecific flulike prodrome that is followed by a rapid evolution to full-blown bacteremic septic shock — accompanied by hypotension, anuria, and clinical evidence of disseminated intravascular coagulation — thus making this syndrome a true medical emergency. The subsequent clinical course often mirrors that of the Waterhouse-Friderichsen syndrome, with bilateral adrenal hemorrhages noted at autopsy.
• Despite appropriate antibiotics and intensive therapeutic intervention, the overall mortality rate in older published studies of established cases of OPSI varied from 50-70%. More recent information suggests that if patients seek medical attention promptly, the mortality rate may be reduced to approximately 10%. Of those patients who die, more than 50% die within the first 48 hours of hospital admission.
• Most instances of serious infection are due to encapsulated bacteria, such as pneumococci (eg, S pneumoniae). Pneumococcal infections account for 50-90% of cases reported in the literature and may be associated with a mortality rate of up to 60%. H influenza type B, meningococci, and group A streptococci account for an additional 25% of infections.
• Possible OPSI involving an asplenic individual constitutes a medical emergency. The critical point in management remains early recognition of the patient at risk, followed by subsequent aggressive intervention. The diagnostic workup should never delay the use of empiric therapy.Possible choices of empiric antimicrobial agents include cefotaxime (adult dose of 2 g IV q8h; pediatric dose of 25-50 mg/kg IV q6h) or ceftriaxone (adult dose of 2 g q12-24h; pediatric dose of 50 mg/kg IV q12h). Unfortunately, some penicillin-resistant pneumococcal isolates are also resistant to cephalosporins. If such resistance is suggested, consider using vancomycin.
• The precise incidence of OPSI remains controversial. Overall, the most reliable data related to incidence estimate approximately 1 case occurring per 500 person-years of observation. Asplenic children younger than 5 years, especially infants splenectomized for trauma, may have an infection rate of greater than 10%.
• Splenectomy performed for a hematologic disorder, such as thalassemia, hereditary spherocytosis, or lymphoma, appears to carry a higher risk than splenectomy performed as a result of trauma. A major contributing factor is the frequent existence of splenic implants or accessory spleens in traumatized patients.
• Preventative strategies for OPSI fall into 3 major categories: education, immunoprophylaxis, and chemoprophylaxis.
• Education represents a mandatory strategy in attempting to prevent OPSI. Asplenic patients should be encouraged to wear a Medi-Alert (Pinellas Park, Fla/Henderson, Nev) bracelet and carry a wallet card explaining their lack of a spleen. Patients should also be aware of the need to notify their physician in the event of an acute febrile illness, especially if it is associated with rigors or systemic symptoms.
• An appropriate factor in preventing OPSI entails vaccination. This has best been defined for S pneumoniae. Unfortunately, the most virulent pneumococcal serotypes tend to be the least immunogenic, and evidence indicates that the efficacy of the vaccine is poorest in younger patients, who would be at higher risk. However, under ideal conditions in a healthy immunocompetent host, the vaccine offers a 70% protection rate.The pneumococcal vaccine should be administered at least 2 weeks before an elective splenectomy. If the time frame is not practical, the patient should be immunized as soon as possible after recovery and before discharge from the hospital.
• Most authorities recommend antibiotic prophylaxis for asplenic children, especially for the first 2 years after splenectomy. Some investigators advocate continuing chemoprophylaxis in children for at least 5 years or until age 21 years. However, the value of this approach in older children or adults has never been adequately evaluated in a clinical trial.

Surgical Care

Splenic trauma is the most common indication for splenectomy, although attempts at splenic preservation are increasingly important. Nonsurgical management for splenic trauma has success rates of 52-98%, with failure usually occurring in the first 96 hours. Splenic cysts, tumors, and vascular lesions may also require surgical removal. Whenever possible, splenic tissue is preserved to decrease the risk of septicemia, but total splenectomy is occasionally necessary. Splenectomy can cure hypersplenism but is not usually indicated because the secondary cytopenias rarely cause serious problems. However, in patients with portal hypertension, vascular shunts may be necessary to prevent esophageal variceal bleeding.

• Splenectomy may be helpful in improving cytopenias in several medical conditions, including congenital anemias (eg, hereditary spherocytosis, elliptocytosis) and autoimmune disorders (eg, immune thrombocytopenic purpura, autoimmune hemolytic anemia, hypersplenism). In thalassemia major, splenectomy may initially decrease the transfusion requirements caused by hypersplenism. However, the benefit must be carefully weighed against the risk of sepsis.
• In Gaucher disease, splenectomy may be necessary when the mechanical strain of the enlarged spleen requires intervention.
• Splenectomy may be indicated in children with sickle cell anemia and a history of splenic sequestration crisis in order to prevent recurrences of the crisis.
• As part of exploratory laparotomy, splenectomy was once an important component of staging of Hodgkin disease. This procedure is rarely used because of improvements in imaging modalities, the high risk of postsplenectomy sepsis, and the increased use of chemotherapy in patients, which allows treatment decisions to be made on the basis of radiologic evaluation alone. Furthermore, data suggest that splenectomy increases the risk of second malignancy in patients treated for Hodgkin disease.