PRESENTATION Albert Chow, MD Pediatric Rheumatology Fellow - - PowerPoint PPT Presentation

FELLOW CASE PRESENTATION

Albert Chow, MD Pediatric Rheumatology Fellow 4/29/2017

Disclosures

• I have no financial disclosures.

Objectives

• Present a rare rheumatology case that can be seen in

pediatric and adult patients

• Discuss pathologic findings
• Discuss potential associations between case and other

rheumatologic conditions

Case – Timeline to Presentation

4/10 5/2 7/4 7/9 7/11 7/15 7/25 7/26 8/1 8/7

Case

• PMHx:
• Unremarkable
• FHx:
• SLE in a second cousin
• T2DM in maternal grandma, maternal aunt, maternal uncle
• Thyroid cancer in a great maternal uncle
• SHx:
• Is a labor worker with his step-father
• Worked blueberry fields last summer
• Currently working on home improvement – painting, masking,

caulking, etc

• Went hiking at Little Mountain in early July 2016

Initial Labs

WBC 1.7 Abs Neutrophils 1139 Abs Bands 255 (15%) Abs Lymphocytes 469 Hemoglobin 11.9 Hematocrit 43.9 Platelets 75 CRP 1.9 ESR 26 D-dimer 1.9 PT / PTT / INR Normal Urinalysis Normal

Initial Exam

General Ill-appearing, shaking with chills HEENT Numerous small lymph nodes along anterior and posterior cervical chains, and along clavicle CV Periods of hypotension to 90s/40s Abdomen Diffusely tender, liver and spleen edges palpated at 1 cm below costal margins Neuro Photophobia MSK Prominent right 3rd PIP but no joint effusions noted, mildly limited ROM of fingers that may be his baseline Skin No rash or lesions

ID Consult

• Recommendations:
• EBV, CMV
• HIV
• TB
• Respiratory viral panel
• Urine GC/CT
• Parvovirus B19
• Lipase
• Uric acid, LDH, ferritin
• Consider abdominal

imaging

EBV IgM (-), IgG (+) CMV IgM (-), IgG (+) HIV Negative TB Negative Resp viral panel Negative Urine GC/CT Negative Parvo B19 Negative Lipase 533

Rheum / HemeOnc / GI Consults

Ferritin 4280 Triglycerides 104 LDH 3732 Uric acid 2.9 Fibrinogen 439 → 177 D-dimer 1.9 → 2.6 ANA 1:80 dsDNA 3 ANCA Negative C3 117 C4 37 AST 409 ALT 452 CK 118 Aldolase 10 Albumin 2.8 Lipase 525 Amylase 178

Additional Studies

• CT Abdomen with contrast: normal
• CT Neck/Thorax with contrast: “Mildly enlarged axillary

lymph nodes bilaterally. This finding is overall nonspecific, and may be related to reactive adenopathy, a systemic inflammatory process, or underlying lymphoproliferative disease.”

• Echo: normal

Pathology

• Bone marrow biopsy:
• “The etiology of the patient's pancytopenia is not clear.

Hemophagocytosis cannot be adequately evaluated due to inadequate aspirate sample. The biopsy shows moderate increase

• f CD163 positive histiocytes/monocytes. There is no evidence of

EBV in the marrow. Close clinical follow up is recommended. See separate report on flow (CM-16-1780).”

Pathology

• Axillary lymph node biopsy:
• “The histologic features are that of necrotizing lymphadenitis, and

with the MPO positivity in the crescentic histiocytes and the CD123 plasmacytoid dendritic cells, is consistent with Kikuchi disease. There is no evidence of infection, as there are no neutrophils or

• granulomas. The EBER positivity is consistent with past infection.

In the differential with Kikuchi disease is systemic lupus erythematosus which can have the same necrotizing lymphadenitis histology findings. Clinical correlation and follow up is recommended.”

Kikuchi Disease

• Also called Kikuchi-Fujimoto Disease
• Affects women more than men
• Clinical features:
• Fever (1 week to 1 month)
• Cervical lymphadenopathy
• Night sweats
• Nausea / Vomiting
• Weight loss
• Diarrhea

Kikuchi Disease

• Kucukardali et al. “Kikuchi-Fujimoto Disease: analysis of

244 cases.” Clin Rheum. 2007; 26(1):50.

• Lymphadenopathy

100%

• Leukopenia

43%

• High ESR

40%

• Fever

35%

• Anemia

23%

• Rash

10%

• Fatigue

7%

• Joint pain / Arthritis

7%

• Hepatosplenomegaly

3%

Kikuchi Disease

http://medpics011.blogspot.com/2014/01/kikuchi-disease.html http://www.pathpedia.com/education/eatlas/histopathology/lymph_ node/kikuchi-fujimoto_disease.aspx

Kikuchi Disease – Pathology

• Histiocytic necrotizing

lymphadenitis

• There is presence of

necrosis without neutrophils

• There may be crescentic

histiocytes

• Immune response of T

cells and histiocytes suggest an infectious trigger

Kikuchi Disease – Pathology

• There is some overlap in

histology of Kikuchi disease and SLE

• Histiocytes stain positive

for MPO

• Plasmacytoid dendritic

cells stain positive CD123

• But plasma cells more

commonly stain positive for hematoxylin bodies in SLE

Kikuchi Disease & SLE

Study Location # Cases SLE ANA Dumas et al. “Kikuchi-Fujimoto Disease: Retrospective study of 91 cases and review of literature.” Medicine. 2014; 93(24): 372-82. France 91 25% 45% (>1:320) Kucukardali et al. “Kikuchi- Fujimoto Disease: analysis of 244 cases.” Clin Rheum. 2007; 26(1):50. Turkey 244 13% 7% Kim et al. “Characteristics of Kikuchi-Fujimoto disease in children compared with adults.” Eur J Pediatr. 2014; 173: 111- 16. Korea 140 0% 17% (1:40 – 1:80) >1:320 rare in children

Case – Course & Follow-up

• Hospital Day 4: Started anakinra daily
• Hospital Day 10: Started prednisone
• Hospital Day 13: Stopped anakinra
• Hospital Day 14: Discharged home on prednisone
• 2 months later (10/21/16): Tapered off prednisone