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The cell of origin for this disease is an immature cytotoxic T-cell clonally expressing the γδ T-cell receptor.The disease is seen more often in immunosuppressed recipients of solid organ transplants, an association that has led to the hypothesis that long-term immune stimulation in the setting of immunosuppression is the causative agent.
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 ...
T-cell-prolymphocytic leukemia (T-PLL) is a mature T-cell leukemia with aggressive behavior and predilection for blood, bone marrow, lymph nodes, liver, spleen, and skin involvement. [1] T-PLL is a very rare leukemia, primarily affecting adults over the age of 30. It represents 2% of all small lymphocytic leukemias in adults. [2]
Splenomegaly can result in hematologic disturbances and abdominal pain and can increase the risk for splenic rupture, which also causes spleen pain, from blunt trauma, resulting in life-threatening internal bleeding. [2] Many conditions can cause splenomegaly, such as various infections, liver disease, and cancer. [2]
Enlarged spleen due to myelodysplastic syndrome; CT scan coronal section, spleen in red, left kidney in green. Signs and symptoms are nonspecific and generally related to the blood cytopenias: Anemia (low RBC count or reduced hemoglobin) – chronic tiredness, shortness of breath, chilled sensation, sometimes chest pain [6]
Hepatosplenomegaly (commonly abbreviated HSM) is the simultaneous enlargement of both the liver (hepatomegaly) and the spleen (splenomegaly).Hepatosplenomegaly can occur as the result of acute viral hepatitis, infectious mononucleosis, and histoplasmosis or it can be the sign of a serious and life-threatening lysosomal storage disease.
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Desmoplastic small-round-cell tumor (DSRCT) is an aggressive and rare cancer that primarily occurs as masses in the abdomen. [4] Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large intestine, small intestine, bladder, brain, lungs, testicles, ovaries, and the pelvis.