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Haematopathology 2016 Craig Soderquist MD and Vinodh Pillai MD PhD Kikuchi disease presenting as abdominal mass Case number Clinical History 8 year old African female Adopted from Ghana no known family history ID: 927642

abdominal mass ihc biopsy mass abdominal biopsy ihc cells adjacent necrosis histiocytes amp positive kikuchi mpo clinical negative cd123

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Slide1

European Association for Haematopathology 2016

Craig Soderquist, MD and Vinodh Pillai, MD, PhD

Kikuchi disease presenting as abdominal mass

Case number

____

Slide2

Clinical History8 year old African female Adopted from Ghana, no known family historyNo past medical history

Chief Complaint: Intermittent fever of 1-2 month duration Unintentional 8 pound (~10%) weight loss

Slide3

Clinical HistoryCBC: WBC: 3.5 K/µl, Hgb

10.2 g/dl, Platelet 226KLabs:Negative infectious workupNegative autoimmune workupTreatment:Antibiotics (cefnidir, amoxicillin, doxycycline)NaproxenPrednisone taper

Slide4

Clinical HistoryImaging: Mesenteric mass (5 cm) abutting the inferior vena cava

Abdominal mass resection highlighted in the following slides

Slide5

Abdominal mass biopsy, H&E, 5x

Slide6

Abdominal mass

biopsy, H&E, 10x

Slide7

Abdominal mass

biopsy, H&E, 40x

Slide8

Abdominal mass

biopsy, IHC, 10xClusters of plasmacytoid dendritic cells adjacent to necrosis CD123

Slide9

Abdominal mass

biopsy, IHC, 20xClusters of plasmacytoid dendritic cells adjacent to necrosis CD123

Slide10

Abdominal mass

biopsy, IHC, 5xMPO positive histiocytes adjacent to necrosis MPO

Slide11

Abdominal mass

biopsy, IHC, 10xMPO positive histiocytes adjacent to necrosis MPO

Slide12

Abdominal mass

biopsy, IHC, 20xMPO positive histiocytes adjacent to necrosis MPO

Slide13

Abdominal mass

biopsy, IHC, 10xCD15 highlights maturing myelomonocytic cells CD15

Slide14

Abdominal mass

biopsy, IHC, 40xCD15 highlights histiocytes

CD15

Slide15

Ancillary StudiesImmunohistochemistry- Unremarkable B and T cells, EBER and ALK-1 are negative.

Flow cytometry Polytypic mature B cells and unremarkable T cells with no aberrant antigen expression or lossCytogenetics and SNP arraysUnremarkableIGH and TCR rearrangement studies: Negative for a clonal population

Slide16

Interesting Features of CaseLarge 5cm mesenteric mass

Significant systemic symptoms for 2 monthsResection showed histiocytic necrotizing lymphadenitisOverall morphological and immunophenotypic features favor kikuchi-fujimoto lymphadentitis

Histiocytic disorder of unknown etiology

Slide17

Proposed DiagnosisHistiocytic necrotizing lymphadenitis, favor kikuchi-fujimoto lymphadenitis