Figure 4
Figure 4. More illustrative examples of FDG-PET and CT scan appearances of some ALPS-FAS patients show splenomegaly and lymphadenopathy. Patient 004 was a 25-year-old man who presented with fever, mouth ulcers, and neutropenia; suspected lymphoma was ruled out after these scans and biopsy of cervical lymph node. The patient underwent splenectomy for persistent cytopenias. Patient 072 was a 12-year-old asymptomatic boy with cervical and axillary adenopathy. Patient 161 was a 9-year-old boy treated with MMF for chronic cytopenias for the last 8 years; 3-dimensional CT reconstruction shows enlarged spleen with volume measured at 1972 cm3. Patient 317 is a 22-year-old man with splenomegaly; CT scan shows a spleen spanning 27 cm. Bottom panel: Chest CT scan appearance in a 19-year-old man (ALPS patient 109) with otherwise asymptomatic nodular lymphocytic pulmonary infiltrates. This patient has also been on therapy for his chronic cytopenias with MMF for the last 7 years.

More illustrative examples of FDG-PET and CT scan appearances of some ALPS-FAS patients show splenomegaly and lymphadenopathy. Patient 004 was a 25-year-old man who presented with fever, mouth ulcers, and neutropenia; suspected lymphoma was ruled out after these scans and biopsy of cervical lymph node. The patient underwent splenectomy for persistent cytopenias. Patient 072 was a 12-year-old asymptomatic boy with cervical and axillary adenopathy. Patient 161 was a 9-year-old boy treated with MMF for chronic cytopenias for the last 8 years; 3-dimensional CT reconstruction shows enlarged spleen with volume measured at 1972 cm3. Patient 317 is a 22-year-old man with splenomegaly; CT scan shows a spleen spanning 27 cm. Bottom panel: Chest CT scan appearance in a 19-year-old man (ALPS patient 109) with otherwise asymptomatic nodular lymphocytic pulmonary infiltrates. This patient has also been on therapy for his chronic cytopenias with MMF for the last 7 years.

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