Clinical Case Conference Ravi Maddipati September 29, 2010 History • CC: Bilateral Lower Extremity Edema • HPI: 61M h/o Stage IVa Follicular lymphoma, HTN, DM, CAD s/p CABG, EtOH cirrhosis, p/w BLE • Edema was consistent with prior venous stasis for which he was taking lasix • On arrival to the floor patients edema was improved but a new supraclavicular node was found. • Reports h/o Stage IVa follicular lymphoma w/ mediastinal, peritoneal, axillary LN, and BM metastases. Diagnosed in May but had not initiated chemo 2/2 questionable HBV status • Patient was seen by Heme-Onc who wanted to initiate RCHOP ASAP but due to black box warning about rituximab and HBV they needed clarification of patients HBV status and possible management options while on chemo. History • PMH: – Stage IVa follicular lymphoma. Diagnosed in 5/2010 with mediastinal, peritoneal, axillary, and Bone marrow metastases. – CAD s/p CABG – EtOH cirrhosis but improved with alcohol abstinence. No Biopsy performed – Persistently elevated anti-HBc IgG since 2007 with rest of panel negative – HTN, HL, DM2, PTSD • SH: – EtoH: Stopped 2mo ago but used to drink 1pt Jack Q2 days – Drug: Stopped crack cocaine 2 months ago. No IVDU • FH: – Sister with diabetes • • • ROS: increasing neck swelling, occasional chills, weight loss NKDA Meds: – – – – – – – Isordil Sertraline Prazosin Insulin Metoprolol Glipizide Lasix Physical • Gen: Lying comfortably in bed, NAD, A&O X 3 • HEENT: PERL, sclera anicteric, EOMI • Neck: JVP at 10cm, 1cm fixed L supraclavicular LN, no thyroid enlargement • Chest: CTAB, 3cm fixed R axillary LN • Card: RRR, III/VI SEM loudest at RUSB • Abd: Soft, NT/ND, normal bowel sounds, liver edge 2cm below costal margin • Extr: 2+ BLE edema w/ mild erythema • Neuro: non-focal Labs • CBC: 2.5/12.6/41 • CMP: 140/3.5/105/27 BUN/Cr: 6/0.65 Glc: 138 • ALT/AST: 121/65 Aphos: 350 TB/DB: 2.3/1.4 Alb: 3.6 • PTT/INR: 42/1.24 • Hepatitis Panel: HAV immune, HCV negative – HBV: cAb+(IgG+, IgM-), sAg-, sAb-, eAg-, eAb-, DNA- Discussion • What is the HBV status of this patient? • What is the best HBV management strategy while patient is on Rituximab? Hepatitis B • Leading cause of Cirrhosis and HCC in the world • Nearly 400million people are HBV carriers worldwide • 75% of those are from Asia and Pacific Islands – Lifetime risk of HBV is 60-80% in highly endemic regions – Majority of transmission is vertical, especially in HBeAg +mothers • US HBV rates declining due to vaccination, sex education, blood product screening – 78,000 new cases/year, mainly in 20-29y.o. – 40% heterosexual contact, 20% IVDU, 12% MSM – Chronic HBV in 0.2-0.3% of US population • HBV is highly infectious, even more so than HCV and HIV – HbeAg + indicates higher risk of transmission • Blood transfusions rare cause of HBV now due to effective screening methods – Isolated anti-HBc + blood also excluded due to low levels of HBV DNA. HBV Genotypes Geographic Distributions A: Northwestern Europe, North America, Central Africa B: Southeast Asia, including China, Japan, and Taiwan (prevalence is increasing in North America) C: Southeast Asia (prevalence is increasing in North America) D: Southern Europe, Middle East, India E: West Africa F: Central and South America, United States (Native Americans), Polynesia G: United States, France H: Central and South America Proposed Clinical Associations Time to HBeAg seroconversion and probability of HBsAg loss: B < C Response to treatment with interferon-α: A > B ≥ C > D Precore/core promoter mutant frequency: precore mutation not selected with A and F Liver disease activity and risk of progression: B < C Evolution to chronic liver disease: A < D Hepatocellular carcinoma risk: B > C in younger age group in Taiwan but B < C in older age group in Japan Mechanism of liver injury • HBV is generally not a cytopathic virus, and severity of liver disease is related to intensity of host immune response • Initial response involves both innate and adaptive immune system to help clear the virus • CD8+ T-cells are activated by presentation of virus in lymphoid organs and are mainly responsible for clearing the virus in acute infection. • Neutralizing antibody production limits intra-hepatic spread of virus and prevents re-infection • In chronic HBV majority of response is mediated in a non-antigen specific manner. HBV mutations • HBV virus replication is highly replicative with low fidelity leading to many mutations • Surface antigen: mutations in antibody-binding domain but clinical significance is controversial • Pre-core, Basal Core promoter, and Core genes: mutations result in decrease of HBeAg – Pre-core mutations result in abolishing HBeAg synthesis while core promoter mutations decrease production by 70% – Associated with cases of fulminant hepatitis, blocking recognition of HBV by CTL’s thus decreasing viral clearance, and poor response to IFN therapy • Polymerase: mutations are often associated with nucleos(t)ide analog resistance – YMDD mutants have marked resistance to Lamivudine. – Lamivudine resistance is 20% at 1 year and 70% at five years. Adefovir is 3% at two years and 29% at five years. Entecavir is only 1% at five years – Cross resistance exists among Lamivudine, Telbivudine, and Entecavir but entecavir requires additional mutations so resistance is less. Natural History Infection with HBV Chronic HBV Acute HBV Immunotolerant Clearance of virus Fulminant hepatic failure Immunoactive Inactive Carrier Resolution Clinical features of Acute HBV • Age at which someone is infected is the principal determinant of clinical outcome – Infected Neonates: 95% will develop Chronic HBV – In adults only 1-5% will develop Chronic HBV. Most clear the infection • Less than 1% of infected adults will go on to develop fulminant hepatic failure. – If liver failure ensues there is only a 20% survival rate without transplant and 50-60% with transplant. – Patients >40 years of age are more susceptible • Usually clinical symptoms include: – Serum sickness like prodrome (fever, arthralgia, and rash) due to immune complex activation of complement. – ALT/AST in the 1000-2000 range. Jaundice occurs in 30% of patients. – Symptoms are self-limited and resolve with clearance of infection Chronic HBV Chronic HBV Check eAg, eAb, DNA, ALT eAg+, eAb- eAg-, eAb+ Immunetolerant or Active ALT >2xULN DNA>20K Y Inactive Carrier or eAg- Hepatitis ALT >2xULN DNA>20K Immunoactive Y N Immunotolerant eAg- Hepatitis N ALT nml DNA<2K N ALT 1-2xULN DNA 2K to 20K Inactive Carrier Q3 ALT x3 then Q6-12 Monitor ALT & DNA q36mo and eAg q6-12mo Q3mo ALT & DNA Treat Y DNA >20K ALT normal ALT 1-2XULN &DNA>20K or Age >40 and DNA >20k Liver Biopsy Inflam Persistent elevation Marker for response to treatment Virologic response Decrease in serum HBV DNA level to <105 copies/mL or <20,000 IU/mL in HBeAg-positive cases and <104 copies/mL or <2000 IU/mL in HBeAg-negative cases Loss of HBeAg with or without seroconversion to anti-HBe Biochemical response Normalization of serum ALT levels On-treatment response Initial response Suppression of HBV DNA levels to <104-5 copies/mL with or without loss of HBeAg, in addition to normalization of serum ALT levels Maintained response Requiring continuation of therapy Off-treatment response Sustained response Virologic and biochemical response observed for 6-12 months after treatment is discontinued Durable response Indefinite virologic and biochemical response after treatment is discontinued Treatments • • • • • • • • • • Peg-IFN Lamuvidine Adefovir Entecavir Tenofivir Telbuvidine Emtricitabine Celvudine Truvada IFN+LAM Isolated anti-HBc positivity • Window period of acute hepatitis B, when anti-HBc is predominantly of the IgM class • Many years after recovery from acute hepatitis B, when anti-HBs has fallen to undetectable levels • False-positive serologic test result • Years after chronic HBV infection, when the HBsAg titer has fallen below the level of detection • HBV-infected persons who are co-infected with HCV • Rarely as a result of varying sensitivity of HBsAg assays. Rituximab and HBV • “Reactivation of Hepatitis B virus after Rituximab-containing Treatment in Patients with CD20-Positive B-Cell Lymphoma”. Matsue K, Kimura S, Takanashi Y, Iwama K, Fujiwara H, Yamakura M, Takeuchi M. Cancer. July 1, 2010 – Determine the rates of reactivation of HBV in patients treated with Rituximab – Retrospective investigation of HBV reactivation after rituximab-containing chemotherapy in 252 B-cell lymphoma patients admitted to a Japanese hospital over a period of five years • Methods: – 252 consecutive patients between April 2004 and April 2009 who were receiving rituximab contacting chemo were enrolled and follow-up occurred for at least 2mo post treatment – All patients were screened at time of admission with HBsAg, anti-HBc, and antiHBs. • If + for anti-HBc then IgM class was checked • If HBsAg was positive during treatment course, HBV-DNA was checked – HBV hepatitis was defined as 2 consecutive ALT >3xULN measured >5 days apart – HBV reactivation defined as seroconversion from sAg neg to sAg pos +/- Rise in DNA Conclusions • HBV reactivation dose occur in the setting of treatment with Rituximab • Isolated Anti-HBc positivity seems to confer the greatest risk to eventual reactivation • Treatment with Entecavir is effective and safe to administer once HBV reactivation is detected • No incidences of Fulminant Hepatic failure were found. • “Prospective Analysis of Heaptitis B virus Reactivation in Patients with Diffuse Large B-Cell Lymphoma after Rituximab Combination Chemotherapy”. Nitsu N, Hagiwara Y, Tanae K, Khori M, Takahashi N. J. of Clin. Oncology. 2010 Sept 13 – 314 patients negative for HBsAg treated fro DLBCL with Rituximab – 16% were found to be HBV carriers and reactivation occurred in 12% with 50% of those being solely anti-HBc+. – Entecavir was initiated when HBV DNA became positive. DNA returned neg. in 13wks. – Risk factors for HBV reactivation were being male and having low anti-HBs titer • “Management of Hepatitis B Virus Reactivation in Patients with Hematological Malignancies Treated with Chemotherapy”. Francisci D, Falcinelli F, Schiaroli E, Capponi M, Belfiori B, Flenghi L, Baldelli F. Infection 2010; 38: 58-61 – 318 consecutive patients undergoing chemo were screened for HBV – HBsAg+ ( 20%), Anti-HBc+/sAb- (19%), Anti-HBc+/sAb+ (61%) – Those with sAg+ were prophylaxed 1wk before chemo and 6mo after chemo with either adefovir, Lamivudine, or combo of the two. DNA became negative after 6 months of treatment in 9/12 patients. – Of those with anti-HBc+ only, were monitored Q1-3mo with serologies. 3/13 became DNA+ and sAg+. Treatment with Adefovir was begun at this time and all achieved virologic and biochemical. Guidelines for HBV carriers who are initiating immunosuppressive therapy • HBV testing should be performed in those at high risk for HBV prior to initiating chemotherapy/immunosuppressive treatment • Prophylaxis is recommended at the initiation of chemotherapy • For VL<2000 anti-viral should be continued for 6mo post chemo • For VL>2000 anti-viral should be continued until they reach same endpoints as in immunocompetent’s • Lamuvidine or Telbuvidine can be used for courses lasting <12mo but for longer courses Entecavir or Tenofovir are preferred • IFN should be avoided Patient Follow-up • Likely recovery from acute hepatitis B(many years ago) vs. Chronic HBV with waning titers of sAb • Patients HBV status should not delay initiation of urgent Chemo • Considering underlying cirrhosis and need for long term treatment he should be started on Entecavir 0.5mg QD and continue 6-12mo after Chemo is completed • Monitor LFT’s and HBV serology's closely References • • • • • • • • “Slesienger and Fordtran’s Gastrointestinal And Liver Disease: Pathophysiology, Diagnosis, Management”. Feldman M, Friedman LS, Brandt LJ. 9th Edition. “Chronic Hepatitis B”. Lok ASF, McMahon BJ. AASLD guidelines 2009 “Reactivation of Hepatitis B virus after Rituximab-containing Treatment in Patients with CD20-Positive B-Cell Lymphoma”. Matsue K, Kimura S, Takanashi Y, Iwama K, Fujiwara H, Yamakura M, Takeuchi M. Cancer. July 1, 2010 “Prospective Analysis of Hepatitis B virus Reactivation in Patients with Diffuse Large B-Cell Lymphoma after Rituximab Combination Chemotherapy”. Nitsu N, Hagiwara Y, Tanae K, Khori M, Takahashi N. J. of Clin. Oncology. 2010 Sept 13 “Management of Hepatitis B Virus Reactivation in Patients with Hematological Malignancies Treated with Chemotherapy”. Francisci D, Falcinelli F, Schiaroli E, Capponi M, Belfiori B, Flenghi L, Baldelli F. Infection 2010; 38: 58-61 “Indications for Therapy in Hepatitis B”. Lok ASF, Degertekin B. Hepatology. May 2009 “The Natural History of Chronic Hepatitis B Virus Infection”. McMachon BJ. Hepatology. May 2009 “Reactivation of Hepatitis B”. Hoofnagle, JH. Hepatology. May 2009