Noticed lump in right groin Sept 2014 Painless Nil inflammation or discolourations Nil associated symptoms US guided core biopsy with GP Oct 2014 17x13x21mm lymph node slightly hypervascular ID: 999373
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1. Treatment of Non-Hodgkin’s Lymphoma in a Patient with Acute Intermittent Porphyria
2. Noticed lump in right groin (Sept 2014)PainlessNil inflammation or discolourationsNil associated symptomsU/S guided core biopsy with GP (Oct 2014)17x13x21mm lymph node, slightly hypervascularMicroscopic examination showed features suggestive of low grade follicular lymphoma; nil evidence of metastatic diseaseHistory
3. CT brain, chest, abdo & pelvisNon-contrast due to poor renal functionPathologically enlarged right inguinal lymph node, no other lymphadenopathyNo hepatosplenomegalyHistory
4. PET scan (Nov 2014)Avid lymph nodes in coeliac axisNil distant or extra-lymphatic metastasis Stage II follicular B cell lymphoma below diaphragm BMAT : infiltration with low volume lymphomaWork-up
5. Acute intermittent porphyria – history of three attacks; asymptomatic past 10 yearsCKI – 2010 Cr 119, eGFR 46Hypertension – on candesartan GORD – oesophagitis visualised on recent PET scanVitamin D deficiencyPap smear in March 2013 showed low grade changesPast medical history
6. First episode in 2000Generalised abdominal crampsStabbing pain, relieved by movementBelieved to be triggered by floor board polish??Urine sample turned red overnightHospitalised – initially thought to be constipation/bowel obstructionMx – pethidine and suppositoryPorphyria diagnosis – heme arginate (thrombophlebitis)History
7. Second episode in 2003Triggered by homeopathic agentsThird episode in 2005Triggered by naturopathic agentsNo acute episodes sinceHistory
8. Family history
9. No family history of cancersStrong family history of CVA/IHDMother – CVA and IHD, passed away from STEMIUncle – CVA and IHD, triple CABGMaternal grandmother – CVA and IHDFamily history
10. Home alone with dogMyotherapist and educatorWell supported by extended familySocial history
11. ChemotherapyR-CHOP – 4 cyclesFollowed by maintenance MabtheraManagement
12. Porphyria and Drug InteractionsDiscussion:
13. Porphyria are a group of metabolic disorders caused by altered enzyme activities within the heme synthesis pathway, due to inherited genetic mutation1Heme production Bone marrow (~80%) – prosthetic group for haemoglobin Liver (~20%) – production of cytochrome P450 enzymesOthers (<1%)Porphyria
14. Medication is one of the most important exacerbating factors for porphyria (especially acute intermittent porphyria)Build-up of neuro-toxic porphyrin (any of the heme pathway intermediates) due to upregulation of the heme pathwayPorphyria and Drug Interaction
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16. Porphyria and Drug InteractionExacerbation of porphyrin can be caused byInduction of Cytochrome P450 enzymes (CYP’s)Superfamily of hemoprotein commonly involved in drug metabolism2Induction of hepatic delta-aminolevulinic acid synthetase-1 (ALAS1)Rate-limiting enzyme in the heme synthesis pathway3Transactivation of Pregnane X receptorInduces CYP3A4 in presence of foreign toxic substance4
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18. Implicated Chemotherapeutic AgentsLikely unsafe; consistent evidenceBusulfanProcabazineFlutamideMegestrolChlorambucilLomustine*According to American Porphyria Foundation databasePossibly unsafe; inconclusive evidenceTamoxifenEtoposidePaclitaxelTopotecanIronotecanIdarubicinMitomycinMitoxantroneLetrozoleEstramustineDacarbazineIxabepiloneVinblastineVinorelbineIfosfamide