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Splenic sequestration crisis is a life-threatening illness common in pediatric patients with homozygous sickle cell disease and beta thalassemia.Up to 30% of these children may develop splenic sequestration crisis with a mortality rate of up to 15%.
Increased deoxygenation causes sickling of red blood cells, which adhere to the spleen wall and splenic macrophages causing ischemia. [2] This ischemia can result in splenic sequestration, where large amounts of blood pool in the spleen but do not flow within vasculature. [11]
There is still much debate to whether pulmonary sequestration is a congenital problem or acquired through recurrent pulmonary infection. It is widely believed that extralobar pulmonary sequestrations are a result of prenatal pulmonary malformation while intralobar pulmonary sequestrations can develop due to recurrent pulmonary infections in adolescents and young adults.
Splenomegaly is an enlargement of the spleen. [1] The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen.Splenomegaly is one of the four cardinal signs of hypersplenism which include: some reduction in number of circulating blood cells affecting granulocytes, erythrocytes or platelets in any combination; a compensatory proliferative response in the bone marrow; and the ...
Splenectomy is usually ineffective for the treatment of cold agglutinin disease because the liver is the predominant site of sequestration. However, if the patient has splenomegaly, then the disease may respond to splenectomy. More importantly, a lymphoma localized to the spleen may only be found after splenectomy.
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
Micrograph of the spleen showing darkly stained, spheroid Gamna-Gandy bodies (arrows) outside the vessel wall at the center. Also shown is diffusely scattered, brown, granular hemosiderin pigment (arrowheads), indicating previous hemorrhage (hematoxylin & eosin staining, 40x magnification).
Particularly hazardous is the downward traction of the phrenicocolic ligament (this maneuver may be necessary for the mobilization of splenic flexure). [ citation needed ] This ligament marks the site where the colon exits the peritoneal cavity: the phrenicocolic ligament is thus an important point of intersection of abdominal anatomy and ...