Complications
Due to an acute infection with parvovirus B19.[17] This virus directly attacks erythroid precursors in the bone marrow and results in erythroid aplasia for about 10 days. Patients with a shortened red-cell life span, such as occurs in HS (and other hemolytic conditions), have a rapidly progressive anemia during this period of absent erythropoiesis, and typically present with marked pallor, lethargy, and fever.
The Hb value is usually between 3 and 6 g/dL. Reticulocyte count is typically <1%. During the recovery phase the reticulocyte count increases.
Following recovery from an aplastic crisis, permanent immunity usually results. This infection may unmask hitherto undiagnosed HS in a family.
Any patient with a chronic hemolytic anemia may develop pigment gallstones. The specific risk in HS is uncertain, but it may be as high as 50% by mid-adulthood.
Gallstones are often asymptomatic, but they may cause problems, such as abdominal pain, fatty food intolerance, and severe jaundice due to obstruction of the common bile duct, and (rarely) biliary cirrhosis or carcinoma of the gallbladder.
Increased production of red blood cells secondary to hemolysis in HS may cause increased pressure within the marrow. Compression and expansion of the adjacent bone may occur. This is especially prominent in the maxilla.
The expansion of the maxilla in children with HS and other hemolytic anemias may cause an overbite that requires orthodontic management.
The most serious and feared complication following splenectomy.
It is generally caused by encapsulated organisms, especially Streptococcus pneumoniae. Precautions, such as appropriate presplenectomy vaccinations, antibiotic prophylaxis, and immediate prompt medical attention with febrile illnesses, have reduced this risk, but it must be stressed to patients that this risk exists lifelong.[5][31]
Patients must be warned that for the remainder of their lives they should seek immediate medical attention for any high fever >100.4°F (>38°C). Asplenic people are also at increased risk of babesiosis and infection following dog bites, and are more susceptible to malaria.[30][52]
Limited emerging evidence suggests that people who have undergone splenectomy for HS or any other indication may be at increased risk of vascular complications years after the procedure.[30][51] Splenectomized children have elevated C-reactive protein (CRP) and D-dimer levels and persistent thrombocytosis, which are known risk factors for cerebrovascular and coronary heart disease.[53]
Vascular complications may include pulmonary arterial hypertension, arterial and/or venous thrombosis, or atherothrombosis.[30][51]
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