Table 1
Patient12344
Age67376770
SexFemaleFemaleFemaleMale
MDS typeCMML-1MDS with multi-lineage dysplasia; progression to AMLMDS UnclassifiableMDS with multi-lineage dysplasia
Presenting SymptomsHydronephrosis, abdominal pain, weight lossSerositis, dyspnea, fevers, rashRapidly evolving peri-arteritis, adominal painOrganizing pneumonia, fever, arthralgias
Bone Marrow BiopsyHypercellular, features of CMML-1Normocellular, multilineage dysplasia, 4%blasts, 20q deletionHypercellular, granulocytic hyperplasia, 2% blastsNormocellular, multilineage dysplasia
ANCA and ANANegativeNegativeNegativeNegative
ESR (0–15mm/hr)217657
CRP (<0.9mg/dL)5.431.24.99
Radiographic extent of peri-arteritisInfra-renal abdominal aorta and common iliac arteriesThoracic aorta and branch vesselsCarotid arteries, thoracic and abdominal aorta, femoral vesselsInternal carotid artery
TreatmentCorticosteroidsCorticosteroids, anakinra, methotrexate, IVIGCorticosteroidsCorticosteroids
Clinical CourseUreteral stenting and nephrostomy tube placement improved symptoms. Inflammatory markers remained elevated despite treatment.Multiple treatment modalities failed to improve symptoms or inflammatory markers. MDS transformed to AML requiring stem cell transplant. Her disease was then quiescent.Despite treatment, disease was complicated by aortic dissection which was managed non-operatively. Azacitidine was initiated but she died of infectious complications.Epipharyngeal mass improved with corticosteroids. Disease course was complicated by infection.