Asplenia

• Congenital asplenia is rare. There are two distinct types of genetic disorders: heterotaxy syndrome[1] and isolated congenital asplenia.[2]
• polysplenia

Acquired asplenia occurs for several reasons:

• Following splenectomy due to splenic rupture from trauma or because of tumor
• After splenectomy with the goal of interfering with splenic function, as a treatment for diseases (e.g. idiopathic thrombocytopenic purpura, thalassemia, spherocytosis), in which the spleen's usual activity exacerbates the disease
• After splenectomy with the goal of arresting the progression of cancers (Chronic lymphocytic leukemia,[3] Hodgkin's disease (starting in the 1970s[4]), non-Hodgkin lymphoma[5])
• Due to underlying diseases that destroy the spleen (autosplenectomy), e.g. sickle-cell disease.
• Celiac disease: unknown physiopathology.[6] In a 1970 study,[7] it was the second most common cause of abnormalities of red blood cells linked to hyposplenism, after surgical splenectomy.

Because of the increased risk of infection, physicians administer oral antibiotics as prophylaxis after a surgical splenectomy, or starting at birth for congenital or functional asplenia.

Those with asplenia are also cautioned to start a full-dose course of antibiotics at the first onset of an upper or lower respiratory tract infection (for example, sore throat or cough), or at the onset of any fever. Even with a course of antibiotics and even with a history of relevant vaccination, persons without a functional spleen are at risk for Overwhelming post-splenectomy infection.[23]

In an emergency room or hospital setting, appropriate evaluation and treatment for an asplenic febrile patient should include a complete blood count with differential, blood culture with Gram stain, arterial blood gas analysis, chest x-ray, and consideration for lumbar puncture with CSF studies. None of these evaluations should delay the initiation of appropriate broad-spectrum intravenous antibiotics. The Surviving Sepsis Campaign guidelines state that antibiotics should be administered to a patient suspected of sepsis within 1 hour of presentation. Delay in starting antibiotics for any reason is associated with a poor outcome.[24]

It is suggested that splenectomized persons receive the following vaccinations, and ideally prior to planned splenectomy surgery:

• Pneumococcal polysaccharide vaccine (not before 2 years of age). Children may first need one or more boosters of pneumococcal conjugate vaccine if they did not complete the full childhood series.
• Haemophilus influenzae type b vaccine, especially if not received in childhood. For adults who have not been previously vaccinated, two doses given two months apart was advised in the new 2006 UK vaccination guidelines (in the UK may be given as a combined Hib/MenC vaccine).[25]
• Meningococcal conjugate vaccine, especially if not received in adolescence. Previously vaccinated adults require a single booster and non-immunised adults advised, in UK since 2006, to have two doses given two months apart.[25] Children too young for the conjugate vaccine should receive meningococcal polysaccharide vaccine in the interim.[15]
• Influenza vaccine, every winter, to help prevent getting secondary bacterial infection.

In addition to the normal immunizations advised for the countries to be visited, Group A meningococcus should be included if visiting countries of particular risk (e.g. sub-saharan Africa).[26] The non-conjugated Meningitis A and C vaccines usually used for this purpose give only 3 years coverage and provide less-effective long-term cover for Meningitis C than the conjugated form already mentioned.[27]

Those lacking a functional spleen are at higher risk of contracting malaria,[28] and succumbing to its effects. Travel to malarial areas will carry greater risks and is best avoided. Travellers should take the most appropriate anti-malarial prophylaxis medication and be extra vigilant over measures to prevent mosquito bites.[19]

The pneumococcal vaccinations may not cover some of the other strains of pneumococcal bacteria present in other countries. Likewise, their antibiotic resistance may also vary, requiring a different choice of stand-by antibiotic.

• Surgical and dental procedures - Antibiotic prophylaxis may be required before certain surgical or dental procedures.
• Animal bites - adequate antibiotic cover is required after even minor dog or other animal bites. Asplenic patients are particularly susceptible to infection by capnocytophaga canimorsus and should receive a five-day course of amoxicillin/clavulanate (erythromycin in patients allergic to penicillin).[29]
• Tick bites - Babesiosis is a rare tickborne infection. Patients should check themselves or have themselves inspected for tick bites if they are in an at-risk situation. Presentation with fever, fatigue, and haemolytic anaemia requires diagnostic confirmation by identifying the parasites within red blood cells on blood film and by specific serology. Quinine (with or without clindamycin) is usually an effective treatment.[29]
• Alert warning - People without a working spleen can carry a card, or wear a special bracelet or necklet which says that they do not have a working spleen. This would alert a healthcare professional to take rapid action if they become are seriously ill and cannot notify them of their condition.[30]