PMC6512020_01
PET-CT hypermetabolic splenomegaly, perihepatic hypermetabolic lymph nodes, and cervical hypermetabolism.
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Clinical examination found a huge splenomegaly, confirmed by abdominal CT scan but no arthritis. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18FDG PET-CT) showed an enlarged hypermetabolic spleen and perisplenic and hilar hepatic hypermetabolic lymph nodes with hypermetabolism around the cervix (Figure 1).
Hemophagocytosis at bone marrow aspiration.
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New biological investigations were suggestive of HPS (sCD25 at 15000 pg/mL and ferritin at 1740 mug/L) with evidence of hemophagocytosis at bone marrow aspirates, with acute renal failure and hypopituitarism (Figure 2), clearly concerning gonadotropic axis and thyrotropic axis.
Brain MRI showing enlargement of the pituitary gland and pituitary stalk without evidence of adenoma.
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Brain MRI presented an enlarged pituitary gland and pituitary stalk with nonspecific lesions (Figure 3).
Brain MRI showing left prerolandic lesion and medial medullary hypersignal. (a) Prerolandic lesion with homogeneous corticosubcortical ring enhancement. (b) Medial medullary hypersignal in T2 and flair.
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It showed a hypersignaling (T2/flair) medial medullary lesion associated with corticosubcortical prerolandic homogeneous lesion (Figure 4).