​Kembara Xtra - Medicine - Hypersplenism

​Kembara Xtra - Medicine - Hypersplenism 
Hypersplenism, or excessive spleen activity, manifests as the following symptoms: Splenomegaly, which occurs frequently but not always.
- Cytopenias with corresponding bone marrow hyperplasia of precursors - Splenectomy to resolve cytopenias

Splenomegaly and hypersplenism are not the same thing.
As shown in immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia, the spleen can become overactive without growing larger. Similar to this, hypersplenism is not always present in splenomegaly.

Epidemiology 
In patients with cirrhosis and portal hypertension (HTN), it may be as common as 30–70%. 

Pathophysiology and Etiology 

The sequestration of produced blood components by an enlarged spleen causes peripheral cytopenias and concurrent bone marrow precursor hyperplasia.
● The following list includes several of the typical etiologies. Hypersplenism can be brought on by almost any splenic or hematologic condition: - Contaginous
 Malaria, Brucellosis, and Tuberculosis
Leishmaniasis, Ehrlichiosis, Schistosomiasis, Histoplasmosis, Candidiasis, Viral, Syphilis, and Hematologic Infectious Endocarditis
Polycythemia vera and myeloproliferative diseases
Hematologic malignancies, primary hypersplenism, ITP, and neoplastic hemolytic anemias
Melanoma and several carcinomas
Gaucher disease, storage disorders, and metastatic malignancies
Niemann-Pick illness
Sarcoidosis, Amyloidosis, Congestive Felty syndrome, Systemic lupus erythematosus, and Inflammatory Glycogen Storage Disease
Cirrhosis and cardiac arrest
 Splenic or portal vein thrombosis Congenital portal vein abnormalities

Patients may complain of abdominal fullness or the spleen protruding through the abdominal wall. They may also express early satiety if the spleen is compressing the stomach.
Particularly with viral infections, patients may complain of pain in the left upper quadrant. Spleen enlargement in lymphoproliferative diseases can go unnoticed unless there is splenic infarction. Given that the spleen is situated near to the diaphragm, the phrenic nerve may convey a feeling of fullness to the C3-C5 dermatomes in the left shoulder.
There could be signs of the hypersplenic underlying condition.

Splenomegaly on a clinical examination
The typical spleen cannot be touched. In the proper clinical setting, a palpable spleen always denotes an underlying problem such as splenomegaly, which leads to hypersplenism, or it may denote a wandering spleen. Additionally, spleen can be palpated in cases of acute asthma exacerbation and chronic obstructive pulmonary disease without the presence of splenomegaly.
● Start off by percussion Traube semilunar space, bordered superiorly by the left 6th rib, inferiorly by the left costal margin, and laterally by the left anterior axillary line. Typically, this area is hollow. The percussion in this region may become dull due to splenic enlargement. The dullness may also be brought on by pleural or pericardial effusions. Additionally, if the patient just consumed a substantial meal, this region could be difficult to percussion.
Gently place your hand on the patient's belly while they are supine to avoid any rapid contractions of the abdominal muscles that can make palpation difficult. By flexing the knees in the direction of the abdomen, you can achieve greater abdominal relaxation. The spleen moves medially and caudally as it grows. Beginning with the right lower quadrant, palpate your way to the umbilicus in the left upper quadrant. Ask the patient to take a deep breath, which will move the diaphragm and, in turn, the spleen into the examiner's hands if there is any uncertainty about whether the spleen has extended over the costal margin.
Petechiae, purpura, or ecchymosis: if thrombocytopenia is present; Jaundice: if hemolytic anemia is present or in advanced cirrhosis; Lymphadenopathy: in hematologic or solid organ cancers. It can be observed in infectious etiologies as well.


Laboratory Results 

Any and all cell lines may be lowered on CBC, leading to the following outcomes: 
Blood loss
Thrombocytopenia and leukopenia

Initial tests (lab, imaging): CBC, reticulocyte count, and haptoglobin if anemia is present.
If there is hemolysis, there should also be signs of unconjugated hyperbilirubinemia, an increased reticulocyte count, an increased LDH, and decreased haptoglobin.
US, CT, Tc-99m sulfur colloid scintigraphy, PET, and MRI are all available.

Tests in the Future & Special Considerations

Testing for particular infectious etiologies may be necessary based on additional history and exam findings: 
EBV serologies, HIV ELISA with Western blot, PPD for tuberculosis, HIV blood parasite smear for malaria and other parasitic diseases, JAK2 mutation in polycythemia vera, Hgb electrophoresis in hereditary hemoglobinopathies, and Hgb electrophoresis

Bone marrow biopsy and liver biopsy for cirrhosis and other storage disorders are two diagnostic procedures. Interpretation of Tests
bone marrow precursor hyperplasia, particularly that which is associated with the patient's unique cytopenias


Treatment Patients with hypersplenism cannot be prescribed any specific medicine. Treating the underlying illness is the most crucial intervention.
If ITP is the cause, the following treatments may be helpful for the patient: If an infectious etiology is found, treatment with the proper antibiotic treatments may assist to ameliorate the cytopenias. - Prednisone or methylprednisolone - IVIG - Rituximab.

Surgery: Splenectomy is a common surgical procedure for people with severe, uncontrolled cytopenias; laparoscopic surgery is preferred over open surgery.


Caution 

Pneumococcal, meningococcal, Haemophilus influenzae, and influenza vaccinations should be given to splenectomized patients at least 14 days before the procedure.

Wait at least 14 days after the splenectomy before getting immunized if this can't be done (for example, in cases of emergency splenectomy).
Vaccine against pneumococci
 The pneumococcal polyvalent-23 vaccine (PPSV23) for use in fully immunized adults and children under the age of two
Pneumococcal polyvalent-13 vaccine (PCV13) for infants and early children 2 months of age as part of standard vaccination schedule PCV13 for children >2 years of age, adolescents, and adults in addition to PPSV23. For scheduling of administration, consult the CDC.
Current recommendations call for a single PPSV23 revaccination five years after the first dose and again at age 65, at least five years following the first dose.
- Influenza vaccination
All unvaccinated children under the age of five should receive one dose of the conjugate vaccination against H. influenzae type B (Hib).
Children under the age of 5 should also receive vaccinations.
For timing, consult the CDC.
Additionally, vaccinated people can receive additional vaccination doses.
- Meningitis vaccinationMeningococcal conjugate vaccination (MCV4) recommended for individuals aged 2 to 55 Meningococcal polysaccharide vaccine (MPSV4) for people over 55; recertification is advised every five years.
– Although patients are not at higher risk from influenza itself, infection with influenza may place patients at higher risk for subsequent bacterial infections, hence influenza vaccination should be given annually based on prevalent circulating strains.

More frequently used, radiofrequency ablation (RFA) can successfully stop hypersplenism from returning. Whether there are any differences between RFA and splenectomy in terms of postoperative infection hazards is unknown at this time. Total and partial splenic embolization and shunting are additional options to splenectomy, albeit these procedures are still in development and require further research to assess their efficacy and morbidity in comparison to splenectomy.

Admission 
Hypersplenism by itself typically does not justify admittance. All patients, however, should be constantly watched for cytopenia-related problems, such as bleeding and infection, as well as splenomegaly-related consequences, such as an elevated risk of splenic rupture. Some patients' huge spleens squeeze their stomachs, making it difficult for them to take in enough food orally.
Patients who have had splenectomies are more susceptible to infection and sepsis following the procedure, particularly when Streptococcus pneumoniae is involved. Clinical decompensation can happen within hours if there are fevers, chills, or discomfort indicative of an underlying infection. While the evaluation is being conducted, empiric broad-spectrum antibiotics should not be delayed. The following are examples of typical empiric regimens: Vancomycin 1 g IV q12h and Ceftriaxone 2 g IV q24h Vancomycin 1 g IV q12h and Levofloxacin 750 mg IV q24h in patients with -lactam allergies 

Continuous Care: Adult splenectomized patients should be instructed to keep a watchful eye out for fever or rigors at home, since these symptoms could be a precursor to bacteremia. They should be told to start taking antibiotics right away before going to a hospital for testing. Early use of antibiotics has been demonstrated to lower mortality from severe post-splenectomy sepsis. Despite the lack of controlled trials, some regimens include the following: - 875 mg of amoxicillin-clavulanate PO BID
500 mg of cefuroxime axetil PO BID.
Levofloxacin 750 mg PO or moxifloxacin 400 mg PO daily are two examples of extended-spectrum fluoroquinolones that can be administered to patients who are allergic to beta-lactam antibiotics.
● Up until age 5 or at least three years after splenectomy, daily antibiotic prophylaxis with penicillin VK or amoxicillin is advised in children who have undergone splenectomy to prevent severe post-splenectomy sepsis: Ages 2 months to 5 years: 125 mg PO BID, and older than 5 years: 250 mg PO BID

Patients who have had their spleens removed should receive in-depth counseling on the possibility of developing severe sepsis after having their spleens removed, as well as the importance of getting a quick medical checkup if they have any worrying symptoms like fever or chills.