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I Saved the Best for Last!

I Saved the Best for Last!. Wednesday ID Case Conference Christopher Hurt, MD May 28, 2008. HPI. BLJ is a 62yo BM p/w fever, functional decline over 5-6 months Had mechanical fall @ that time, then “lump” in L neck/jaw, which resolved on its own

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I Saved the Best for Last!

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  1. I Saved the Best for Last! Wednesday ID Case Conference Christopher Hurt, MD May 28, 2008

  2. HPI • BLJ is a 62yo BM p/w fever, functional decline over 5-6 months • Had mechanical fall @ that time, then “lump” in L neck/jaw, which resolved on its own • Loss of appetite, intermittent epigastric pain, NS, and fevers – with ramp-up in prior 6 weeks • Hospitalized for sxs @ McCain Correctional Hospital • Temps to 104.8 • Weight loss from 225 to 185 pounds

  3. HPI, cont’d • Other c/o included subjective tongue enlargement with dysguesia; occasional HA; currently stiff neck • Sore throat during this time, also - with indolent onset of painful R knee swelling occurred during early part of hospitalization @ McCain, resolved • McCain MDs comment on cervical and axillary LAD and delirium during fevers • Imaging showed pansinusitis, so Rx’d with pip/tazo and ciproflox, and doxy for atypicals • No signif improvement

  4. HPI, cont’d • Observed to have cont’d fevers and rigors @ UNC • Many imaging studies obtained • HD#2, MRI/A - opacified mastoids, periventricular WM signal change c/w small-vessel dz, L MCA stenosis • HD#3, MRI spine - abnormally thickened, clumped enhancing nerve roots from L3 to S1 • S/o arachnoiditis or leptomeningeal metastatic dz • Diffusely abnormal BM signal, s/o infiltrative process • HD#8, CT neck - B cervical LAD, asymmetric low-density lesion in L parapharyngeal space

  5. HPI, cont’d • Steroids started empirically because of leptomeningeal findings • When stepped down from methylpred 60 q8h to pred 60 q24h… fevers returned • Over first 10 hospital days, transaminases rose and platelets fell • AST 685, up to 1221 • ALT 861, up to 2051 • Platelets down from 153 to 52

  6. PMHx/PSHx • PTSD • Neuropathic pain from LBP • Tinnitus • Two back surgeries, 1975 and 1976 • LTBI, s/p treatment for 6 months with INH in early 1990s

  7. SocHx • Incarcerated for years, and for couple of months PTA was at McCain in Aberdeen • Previously employed as supervisor for mfg of PC boards (those green things) • Had been in US Army, deployed to Viet Nam • Had Agent Orange exposure while there • 4 cigarettes/day, smoked for 45 years • No EtOH, no illicits

  8. No known CA or rheum dz in family At time of consult: Amox/clav Methylprednisolone Gabapentin Citalopram APAP PRN Scheduled ibuprofen NKDA FHx Meds, Allergies

  9. Gen: Fatigued-appearing, lying in bed, coffee ground emesis HEENT: Icteric sclerae. Sub-cm submandibular and ant cervical LAD. No TM. Chest: CTAB CV: Tachy, regular. No m/r/g. Abd: Obese, soft, RUQ tender Ext: No c/c/e x4. Clubbed digits x10. Skin: Two ecchymoses on abdomen, otw no rashes or petechiae. Neuro: CN II-XII intact. PERRL. 4/5 strength hip flexion. Brisk patellar reflexes. Downgoing Babinski bilaterally. PEx Tm 39.4 HR 66-120 SBP 98-122 RR 20 Sat ~95%RA

  10. Labs ANC 9.4 ALC 2.6 10.9 132 102 8 81 12.5 74 4.1 26 0.8 31.9 Date AST ALT AP LD 8/16 876 653 307 3951 8/18 707 481 277 4299 8/20 552 354 205 2459 8/24 310 381 249 1134 8/28 685 861 256 – 8/30 1286 1946 393 – 8/31 1457 1938 434 2344 Haptoglobin <22 D-dimer 3525 ANA neg TSH 1.02 PSA 0.4 ESR 14 CRP 6.5

  11. Hemophagocytic lymphohistiocytosis (HLH) from Epstein-Barr virus infection

  12. More of the story… • LP for mental status change on 8/19 (HD#6) • Enterovirus, HHV-6, HSV, CMV, VZV all negative • EBV added on 8/20, and was positive <250 copies • 8/22 - peripheral blood EBV checked, 7777 copies • Our favorite Heme attending saw Mr J @ our request: “No need for BMBx!” • 8/30 - 128,067 copies • 9/3 - Heme reconsulted (attdgs changed); BMBx pending… • GCV started along with steroids • 9/11 - 453,492 copies • 9/14 - BMBx done • Hypercellular marrow, with hemophagocytosis • FISH studies for myelodysplasia all normal

  13. More of the story… • 9/14-15, chemotherapy started for HLH • Rituximab, cyclophosphamide, vincristine x2d • 9/18 - EBV viral load 1,784,371 copies • Went on to develop: • Klebsiella pneumonia and Gm negative sepsis • VRE UTI • renal failure • pericardial effusion • product-dependent coagulopathy • Expired on 9/28/07, HD#44

  14. Epstein-Barr virus • Cause of infectious mononucleosis1 • Lab worker accidentally got infected, developed heterophile antibodies (1968) • 25-30% of adolescents, adults with primary EBV infection get IM • Two strains (1 & 2), serologically indistinguishable1 • By adulthood 90-95% of population is seropositive2 • Two waves: ≤5yo, and ~25yo 1 Hurt C, et al. Am J Med 120(10):911e1-e8 2 Mandell, 6th Ed.

  15. Infectious mononucleosis • Subacute onset of pharyngitis • Fevers ≥ 37.5°C • Generalized LAD • Palatal petechiae (up to 25%) • Only 4 signs occur statistically more often in IM: • Palatal petechiae, and inguinal, axillary, and posterior auricular LAD Hurt C, Tammaro DT. Am J Med 120(10):911e1-e8

  16. Hemophagocytic lymphohistiocytosis • Uncommon, but EBV is most frequent trigger • If it is EBV-associated, almost uniformly fatal, usu due to delay in dx • Presents as hyperinflammation • Hyperactive phagocytes • Defective apoptotic triggering • Reduced CTL and NK cell activity • Accumulation of M, T-lymphocytes • Defect triggering HLH can be inherited or acquired • Infection of immune effector cells Van der Woude HJ, et al. Chest 2006;130:291-5

  17. HLH clinical presentation • Prolonged fevers, hepatosplenomegaly, cytopenias • Often preceded by URI or GI infection • Mac-pap rashes, elevated liver enzymes, bilirubin, LDH • Coagulopathy may develop Van der Woude HJ, et al. Chest 2006;130:291-5

  18. HLH diagnostic criteria, 2004 • Revised from original 1991 Histiocyte Society version • Diagnostic criteria (any 5 of the 8), unless molecular diagnosis is c/w HLH (generally only for familial): • Fever • Splenomegaly • Cytopenia involving at least 2 cell lines • Hgb <9, Plt <100, PMNs <1 • Hypertriglyceridemia and or hypofibrinogenemia • Elevated ferritin ≥ 500 µg/L • Serum CD25 ≥ 2400 U/mL • Low or absent NK-cell activity • Hemophagocytosis in BM, CSF, or lymph nodes • No e/o malignancy Henter JI, et al. Pediatr Blood Cancer 2006;48(2):124-31

  19. Viruses Adenovirus CMV Coxsackie Dengue EBV Hepatitis A, B, and C HSV and VZV HIV HHV-6 and -8 Influenza and parainfluenza Measles and rubella Parvovirus B19 Bacteria Brucella and Coxiella Chlamydophila psittaci Ehrlichia and Rickettsia Mycoplasma pneumoniae Orientia tsutsugamushi Salmonella typhi MTB, MAC Any spirochetal disease Fungi Aspergillus, Candida Cryptococcus, Histoplasma Pneumocystis jiroveci Infections known to cause HLH Fisman DN. Emerg Infect Dis 2000;6(6)

  20. Treatment • HLH-94 • Initial 8 week chemotherapy • Etoposide q12h for 2 weeks, then weekly • Dexamethasone taper • Intrathecal MTX if progressive neuro sxs present • If partial response seen, continuation therapy started • Etoposide q other week • Dexamethasone for 3d every second week • Cyclosporine A daily • Antifungal and TMP/SMX prophylaxis recommended Van der Woude HJ, et al. Chest 2006;130:291-5

  21. Treatment, cont’d • HLH-2004 • Dexamethasone for 8 weeks on taper • Etoposide for 8 weeks • Cyclosporine for 8 weeks • Intrathecal methotrexate and oral prednisolone if CNS • Move forward with SCT • Busulfan, etoposide, and cyclophosphamide conditioning for week prior to SCT • CsA thereafter • ATG and metronidazole if URD clinicaltrials.gov, NCT 00426101

  22. The End

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