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Fever of Unknown Origin

Fever of Unknown Origin. Ayesha Kelly & Jen Rochette 6/25/08. 4/28/08. 52yo F with history of mild COPD presents with fever x3 days N/V, abd pain, HA for preceding 3 months Tick exposure 5 days prior to admission Fevers 102-104F beginning 3 days prior to admission

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Fever of Unknown Origin

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  1. Fever of Unknown Origin Ayesha Kelly & Jen Rochette 6/25/08

  2. 4/28/08 • 52yo F with history of mild COPD presents with fever x3 days • N/V, abd pain, HA for preceding 3 months • Tick exposure 5 days prior to admission • Fevers 102-104F beginning 3 days prior to admission • On admission found to have neutropenia (WBC 2.1, ANC 0) • Worked up & treated for rickettsial disease

  3. 6/13/08 • Readmitted from ID clinic for intermittent fever x2 weeks • Also c/o HA, back pain, night sweats, RLQ pain, constipation alternating with diarrhea, nausea, fatigue

  4. PMH • Mild COPD • Colonoscopy 2007: 1 polyp removed • Normal mammogram 2008 • Meds • MVI, B12, prn albuterol • Allergies • Sulfa (reaction unknown)

  5. SH • Lives in Mebane with husband • Filtered well water • Works at UNC Student Stores • Daughter & son-in-law run organic chicken & beef farm • Husband works at golf course & is exposed to chemicals on daily basis • 1 dog, no other close animal contact • No recent travel • 60pack year smoking history, recently reduced to 1-2 cigarettes/day; occasional EtOH, denies other drugs

  6. FH: non-contributory • ROS as per HPI • Increased DOE • Denies LAD, rash, cough, URI symptoms

  7. VS: 39.5, 122/80, 103, 22, 95%RA • Gen: NAD, pallor • HEENT: dry MM, posterior pharynx erythematous • Pulm: CTAB with fair air movement • CV: tachycardic, reg rhythm, no M/R/G • Abd: normoactive BS, tenderness RLQ, no rebound/guarding, no masses, no hepatosplenomegaly

  8. Chem 10: wnl • 2.5>11.3/31.3<423 • ANC 0.1 • UA neg • Utox: + MJ & cocaine • CRP 19, ESR 37

  9. Chest XRay

  10. Abdominal CT Mild inflammatory stranding in the sigmoid colon

  11. Discussion

  12. Old labs: • CSF: OP, cell count, culture, crypto Ag, VDRL, HSV neg • Serum: • Neg: ehrlichia, CMV PCR, HIV, EBV PCR, brucella, francisella • Pos: Parvo c/w past infection, 4-fold increase in RMSF, ANA

  13. New labs: • CSF: OP, cell count, culture, crypto Ag, VDRL, HSV neg • Serum: • fungal cx & blood rare pathogen cx neg to date • ANA, HIV, EBV, CMV, blood cx x2, urine cx neg • Bone aspirate: • AFB cx neg to date • Bone marrow cx neg

  14. Bone marrow aspirate • Maturation arrest in the myeloid line • Mild eosinophilia (7%) • Flow cytometry normal, no monoclonal large granular lymphocytes

  15. Diagnosis and Treatment • Neupogen 480 mcg sc qd to shorten length of neutropenia • Protective precautions for neutropenia (hygiene, mask, avoiding uncooked & unwashed foods, avoiding sick contacts, etc.) • Outpatient EGD to look for gastric ulcers • Outpatient colonoscopy to screen for Clostridium septicum

  16. Cyclic Neutropenia

  17. Cyclic Neutropenia • Rare congenital neutropenia • First recognized in 1910 • Regular oscillations in neutrophils, PLTs, monocytes, eosinophils, lymphocytes, & reticulocytes • Typically 21 day cycles (14-28d) • Neutrophils fluctuate between normal & <500 • Marrow may appear hypoplastic with promyelocyte arrest during periods of neutropenia • See lots of promyelocytes but few more mature cells

  18. Cyclic Neutropenia • Childhood onset • More common • Familial pattern, AD • Symptoms tend to regress after puberty • Adult onset • Associated with clonal proliferation of CD56+ large granular lymphocytes (NK cell LGL leukemia)

  19. Pathogenesis • Defect at level of stem cell • Multiple cell lines • Can be transferred from affected bone marrow donor to recipient • Mutation in Neutrophil Elastase gene • Chromosome 19p13.3 • Several different mutations affecting enzyme’s active site possible • Gain of function • Synthesized primarily at the promyelocytic stage • Hypothesized to cause accelerated apoptosis of developing neutrophil precursors and/or enzymatic antagonism of G-CSF

  20. Diagnosis • Documentation of ANC below 500/uL on at least 3-5 consecutive days per cycle of each of 3 regularly spaced cycles • Monitor neutrophil count 3 times/week for 6-8 weeks

  21. Manifestations • Severe neutropenia 3-5 days during each cycle • Malaise, fever, aphthous stomatitis, LAD, ulcers of GI tract, serious cutaneous/subcutaneous infections • Most common infections: bacterial • Staph spp. • Gram negatives • Association with Clostridium septicum

  22. Management of Infection Risk

  23. Treatment • G-CSF is treatment of choice for adult-onset • Required higher doses compared to pts requiring G-CSF for other reasons • May be augmented when combined with SCF • Neutrophil oscillation may persist but neutropenic periods shortened • Recovery: cell density arises as wave traveling through myeloblasts  promyelocytes  myelocytes  neutrophils

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