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ID Fellows Case Conference
August 25, 2010
Lindley Barbee MD, MPH
A classic presentation of an uncommon disease …except among transplant patients.
An Uncommon Presentation of a Common Disease
Case
67 y M w/ small cell lymphocytic lymphoma/chronic lymphocytic leukemia presents to SCCA for evaluation for transplant
SLL/CLL dx’d in late 2006 with diffuse LAD TX course thus far: rituximab, pentostatine,
cyclophosphamide for three months in early 2007 Rituximab, solumedrol for relapse the following year Rituximab, cytoxan, prednisone in 2009 Since January 2010 – bendamustine, rituximab x 8 cycles July 2010 radiotherapy with gamma knife to abdomen
Background History
PMH Gout Benign adenoma of brain
s/p resection 2000 Pituitary adenoma Melanoma s/p excision TURP for BPH
Family History Father died of colon CA Mother died of liver CA
Medications Allopurinol Levothyroxine Cabergoline Dexamethasone 0.5mg Benadryl prn Marinol prn Compazine prn Ambien prn
Background History
Social History
Born Indiana, lives in Carson City NV
In the Marines lived in Japan and San Diego
Travel – Mexico, Kenya & Tanzania
Retired attorney, has a dog, no other animal exposures. Denies tob, etoh, illicit
Pre-transplant Serologies
HSV+, VZV +, CMV -, toxo -, HIV p24 -
Case
On 7/21 he had last XRT to abdominal mass On 7/23 he and his wife left Reno to drive to
Seattle for his pre-txp appt. During the drive suffered abdominal pain, nausea and vomiting.
On arrival to SCCA, July 27, he is weak and sweaty, c/o abdominal pain, n/v.
Labs reveal Na 120. Pt is admitted to UWMC.
Admission Data
VS: T 36.4 HR 80 BP 140/78 RR 18 100% RA
121 | 88 | 16 / AST 61 ALT 61 AlkPhos 121
4.0 | 21 | 0.8 \
2.99 \ / 54
Serum Osms 250 / 38 \
Una 53 75% polys
18% lymphs
Hospital Course: Days 2 - 4
Hyponatremia attributed to hypovolemia. Tx’d with NS and sodium slowly rises Cort stim is WNL GI is consulted for abdominal pain, now with
constipation – dx: Radiation Enteritis Pt is placed on bowel rest and given
parenteral nutrition and miralax
Hospital Course: Days 6 - 7
Pt develops rash on face on evening of HD6 In the morning, he syncopizes is found to be
hypotensive (SBP 80s) and bradycardic (50s) Started on broad spectrum abx –
Vanco, Imipenem and Cipro Transferred to the ICU; started on pressors
Exam
VS: T 37.4 HR 114 BP 105/60 RR 24 99% 2L GEN: wdwn man uncomfortable, rigoring HEENT: EOMI, small vesicular lesions scattered
on face and neck, neck supple LUNGS: tachypneic, CTAB CV: tachy, regular, no m/r/g EXT: cool extremities, trace edema SKIN: scattered vesicles on face, chest, back
upper arms, upper thighs
Example of Vesicles
Laboratory Data
3.84 33 AST 588 ALT 518
35 Alk Phos 197 T bili 2.4
142 118 35 Amylase 476 Lipase 413
4.1 20 1.4
Vesicle Swab FA: + VZV
Serum VZV PCR: 4 million copies
Diagnosis: Disseminated VZV
with Visceral Involvement
Varicella Zoster Virusin Immunocompromised Host
Primary VZV infection Herpes Zoster Multiple involvement of herpes zoster Disseminated VZV Visceral involvement CNS involvement Rare Triad: Hyponatremia, abdominal pain
and disseminated cutaneous VZV
Epidemiology
17-50% of BMT patients are expected to develop zoster in their post transplant course
Approx. 3.6% of those develop visceral VZV Disseminated zoster occurs in ~2.6% of
PBSCT The triad of hyponatremia, abdominal pain,
and disseminated zoster has been reported in 8 cases, mostly among BMT patients
Case Reports
Ref Age/SexPrimary Disease
TX Clinical sx Day TX Outcome
1 57/M CML Allo BMT Abd pain/Ileus /
low Na9
Acyclovir/ valacyclovir
recovered
2 61/F NHL Auto SCTAbd pain/
low NA10 Acyclovir died day 10
3 38/M CMLAllo unrelated
SCTLow Na/abd pain/ileus
10 Acyclovir/ IVIG recovered
3 32/F ALL Allo sib SCTabd pain/ileus,
low Na7 Acyclovir/ IVIG recovered
4 19/F ALL Allo unrelatedLow Na/Abd
pain/hepatitis/ pancreatitis
13 Acyclovir recovered
7 65/F NHL CHOP/XRTAbd pain/
hyponatremia6 Acyclovir recovered
8 ? NHL Chemo/XRTIleus/abd pain/ hyponatremia
? None recovered
9 50/F AML Auto SCTAbd pain/vomit/ low Na/ hepatitis
7 Acyclovir recovered
Case: Therapy and Outcome
Pt was started on high dose acyclovir at 10mg/kg IV q8H
He was treated for 12 days IV and then transitioned to PO valacyclovir
Lesions have scabbed over, Na is corrected, no further abdominal pain.
Still with fevers and mild elevation of transaminases.
VZV PCR Copies and Log10
PCR LOG10 HD6: 4,000,000 6.6 HD12: 1,500,000 6.2 HD18: 8400 3.9 HD22: 4300 3.6 HD26: 7600 3.9
Summary
VZV is a common infection in transplant patients
Its manifestations vary from shingles to fulminant hepatic failure
Think VZV with hyponatremia and abdominal pain in the right immunocompromised host
References
1. Szabo, Horvath, Seimon and Hughes. Inappropriate antidiuretic hormone secretion, abodminal pain and disseminated varicella zoster virus infection: an unusual triad in a patient 6 months post mini-allogeneic peripheral stem cell tranplant for chronic myeloid leukemia. BMT 2000;26:231-3.
2.McIlwaine, Fitzsimons and Soutar. Inappropriate antidiuretic hormone secretion, abdominal pain and disseminated varicella zoster virus infection: an unusual and fatal triad in a patient 13 months post Rituximab and autologous SCT. Clin. Lab Haem 2001;23:253-254.
3. Au, Ma, Cheng, Ooi, and Lie. Disseminated zoster, hyponatremia, severe abdominal pain and leukemia relapse: recognition of a new clinical quartet after BMT. British J of Dermatology 2003; 149: 862-5.
4. Rau et al. Triad of severe abdominal pain, inappropriate antidiuretic hormone secretion, and disseminated varicella-zoster virus infection preceeding cutaneous manifestations after hematopoietic stem cell transplantation: Utility of PCR for early recognition and therapy. Ped Infect Dis J 2008; 27(3):265-8.
5. Kim et al. Factors influencing varicella zoster virus infection after allogeneic peripheral blood stem cell transplantation: low-dose acyclovir prophylaxis and pre-transplant diagnosis of lymphoproliferative disorders. Trans Infectious Disease 2008; 10: 90-98.
6. Shiley and Blumberg. Herpes viruses in Transplant recipients: HSV, VZV, Human herpes viruses and EBV. Infect Dis Clin N Am 2010; 24: 373-93.
7. Ohara et al. Abdominal pain and syndrome of inappropriate antidiuretic hormone secretion as a manifestation of visceral varicella zoster virus infection in a patient with Non Hodgkins Lymphoma. Am J Hematology 2006.
8. Ingraham, Estes, Bern, DeGirolami. Disseminated varicella-zoster infection with the syndrome of inappropriate antidiuretic hormone. Arch Intern Med 1983; 143(6):1270-1.
9. Vinzio et al. Severe abdominal pain and inappropriate antidiuretic hormone secretion preceeding varicella-zoster virus reactivation 10 months after autologous stem cell transplanation for acute myeloid leukaemia. BMT 2005; 35:525-7