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Clinical Case Conference Shivan Mehta, MD August 11, 2010
Case <ul><li>43M h/o Mulitiple Myeloma, chronic kidney disease presents with 2 weeks of hematochezia. </li></ul>
HPI <ul><li>Initially diagnosed with Multiple Myeloma 1 year prior to presentation. Disease course c/b acute renal failure...
PMH <ul><li>PMH: Multiple Myeloma (IgA monoclonal gammopathy) c/b CKD presenting with renal failure requiring HD and plasm...
Physical Exam <ul><li>VS- T 97.8, BP 102/63, HR 85, RR 16, 99% RA  </li></ul><ul><li>Gen- NAD, Ox3, pleasant, comfortable ...
Labs 140| 106|  36 ------------------< 82 4.5 |  25 | 3.4 8.1 5.8 >-----< 186 24 MCV 88, RDW 16.1 TP 7.4, Alb 3.0 Tbili 1....
Differential Diagnosis <ul><li>What is your differential diagnosis for hematochezia in a patient with multiple myeloma? </...
Differential Diagnosis <ul><li>Ischemic colitis </li></ul><ul><li>Infectious colitis </li></ul><ul><ul><li>CMV, Salmonella...
EGD <ul><li>Normal esophagus </li></ul><ul><li>Mild antritis-> pathology shows mild lymphocytic infiltrate, otherwise norm...
Colonoscopy <ul><li>A large 5cm mass lesion with ulceration was seen in the cecum. </li></ul><ul><ul><li>Small fragment of...
Repeat Colonoscopy (1 month) <ul><li>Colonic mucosal biopsies with focal acute inflammation and granulation tissue formati...
Clinical Course <ul><li>1 month later, patient admitted with multiple episodes of hematochezia. Hemoglobin down to 5.3. St...
Repeat Colonoscopy (#3) <ul><li>There were innumerable medium-sized punched-out ulcers in the rectum, rectosigmoid junctio...
Repeat EGD/Colonoscopy (#4) <ul><li>EGD normal. </li></ul><ul><li>Colonoscopy showed similar ulceration throughout colon w...
Differential <ul><li>With multiple unrevealing biopsies, what do you think is still on the differential for colonic ulcers...
Medication-associated colitis <ul><li>“ Acylovir- induced colitis” </li></ul><ul><li>3 cases of hematochezia within 24 hou...
Medication-associated colitis <ul><li>Case report of MM patient treated with Velcade (26S proteosome inhibitor) presenting...
Intestinal Plasmacytoma <ul><li>Case reports of plasmacytoma in GI tract causing bleeding. </li></ul><ul><li>Extramedullar...
Clinical course <ul><li>Patient’s bleeding stabilized and he was discharged to home with stable hemoglobin. </li></ul><ul>...
EGD/Flex Sig (#5) <ul><li>EGD </li></ul><ul><ul><li>Multiple small patchy areas of irregular erosion in the incisura and a...
Pathology
Diagnosis <ul><li>Amyloidosis of the Colon </li></ul>
Amyloidosis <ul><li>Extracellular deposition of protein fibrils with a  β -sheet fibrillar structure. </li></ul><ul><li>De...
Gastrointestinal Amyloid <ul><li>Amyloid may cause GI symptoms extending from mouth to anus </li></ul><ul><li>Amyloid depo...
GI manifestations <ul><li>Ulcers </li></ul><ul><li>Erosions  </li></ul><ul><li>Polypoid lesions </li></ul><ul><li>Submucos...
Prognosis/ Treatment <ul><li>Median survival for AL amyloidosis less than 2 years, if treated with melphalan and prednison...
Follow-up Course <ul><li>After amyloid diagnosis, pt given Etoposide, Cyclophosphamide, Dexamethasone. </li></ul><ul><li>2...
References <ul><li>Ammar T et al. “Primary Antral Duodenal Extramedullary Plasmacytoma Presenting With Melena” Clinical Ga...
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Mehta Presentation

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Mehta Presentation

  1. 1. Clinical Case Conference Shivan Mehta, MD August 11, 2010
  2. 2. Case <ul><li>43M h/o Mulitiple Myeloma, chronic kidney disease presents with 2 weeks of hematochezia. </li></ul>
  3. 3. HPI <ul><li>Initially diagnosed with Multiple Myeloma 1 year prior to presentation. Disease course c/b acute renal failure requiring short course of dialysis. </li></ul><ul><li>Presented to outpatient GI with intermittent hematochezia x 2 weeks. Reports bright red blood up to 4 times a day on toilet paper and mixed with stool. No melena. Mild abdominal pain. </li></ul><ul><li>ROS significant for 25 lb weight loss, mild abd cramping and pain, no dizziness/lt headedness/chest pain/sob </li></ul>
  4. 4. PMH <ul><li>PMH: Multiple Myeloma (IgA monoclonal gammopathy) c/b CKD presenting with renal failure requiring HD and plasmapheresis. Bone marrow biopsy/ fat pad neg for amyloid. </li></ul><ul><li>PSH: None </li></ul><ul><li>Allergies: NKDA </li></ul><ul><li>Meds: Velcade/Decadron/Cytoxan (1 course 3 months prior), Renagel 800mg tid, Nephrocap 1tab daily, Acyclovir </li></ul><ul><li>SH: Works as office manager. Married with no children. Social Etoh. Denies tobacco/illicits </li></ul><ul><li>FH: no malignancy or other GI issues </li></ul>
  5. 5. Physical Exam <ul><li>VS- T 97.8, BP 102/63, HR 85, RR 16, 99% RA </li></ul><ul><li>Gen- NAD, Ox3, pleasant, comfortable </li></ul><ul><li>HEENT- anicteric, pale conjunctivae </li></ul><ul><li>CV- RRR, no m/r/g </li></ul><ul><li>Chest- CTA b/l </li></ul><ul><li>Abd- soft, ND, + BS, mild diffuse TTP </li></ul><ul><li>Ext- 1+ lower extremity edema </li></ul><ul><li>Rectal- no masses, brown stool with streaks of red blood </li></ul>
  6. 6. Labs 140| 106| 36 ------------------< 82 4.5 | 25 | 3.4 8.1 5.8 >-----< 186 24 MCV 88, RDW 16.1 TP 7.4, Alb 3.0 Tbili 1.0, Alk 73 ALT 45, AST 38 PT 13.2, INR 1.1 PTT 26.4 LDH 90
  7. 7. Differential Diagnosis <ul><li>What is your differential diagnosis for hematochezia in a patient with multiple myeloma? </li></ul>
  8. 8. Differential Diagnosis <ul><li>Ischemic colitis </li></ul><ul><li>Infectious colitis </li></ul><ul><ul><li>CMV, Salmonella, Shigella, Campylobacter, E. Coli, Entameoba </li></ul></ul><ul><li>Diverticulosis </li></ul><ul><li>AVM </li></ul><ul><li>Upper GI bleed </li></ul><ul><li>Neoplasm </li></ul><ul><ul><li>Adenocarcinoma, Lymphoma, Plasmacytoma </li></ul></ul><ul><li>Colonic ulcers </li></ul><ul><ul><li>Medications, Idiopathic </li></ul></ul><ul><li>GI amyloid </li></ul><ul><li>Inflammatory bowel disease </li></ul><ul><li>Hemorrhoids </li></ul>
  9. 9. EGD <ul><li>Normal esophagus </li></ul><ul><li>Mild antritis-> pathology shows mild lymphocytic infiltrate, otherwise normal. </li></ul><ul><li>Normal duodenum </li></ul>
  10. 10. Colonoscopy <ul><li>A large 5cm mass lesion with ulceration was seen in the cecum. </li></ul><ul><ul><li>Small fragment of colonic mucosa within normal limits. </li></ul></ul><ul><ul><li>Fragment of fibrinopurulent exudate consistent with nearby ulcer. </li></ul></ul><ul><li>Additional areas of ulceration were seen in the left colon. </li></ul><ul><ul><li>Colonic mucosal biopsies with areas of acute inflammation and ulceration, granulation tissue formation and reactive atypia. </li></ul></ul><ul><ul><li>Underlying homogeneous material favor negative for Congo without typical polarization properties </li></ul></ul>
  11. 11. Repeat Colonoscopy (1 month) <ul><li>Colonic mucosal biopsies with focal acute inflammation and granulation tissue formation. Submucosal pink amorphous material present which stains with Congo red but does not show characteristic apple-green birefringence. </li></ul><ul><li>Addendum: A PAS stain was performed and showed no definitive staining in the amorphous material. A Congo Red stain was repeated and also was negative for apple-green birefringence. Electon microscopy was performed as an aid to evaluate the amorphous material and electron microscopy photographs did not support the presence of amyloid deposition. </li></ul><ul><li>Resolution of large cecal mass with improvement in ulceration throughout colon. Only areas of mild erythema. </li></ul>
  12. 12. Clinical Course <ul><li>1 month later, patient admitted with multiple episodes of hematochezia. Hemoglobin down to 5.3. Stool studies negative. </li></ul><ul><li>CT scan shows: </li></ul><ul><ul><li>Transmural wall thickening with pericolonic fat stranding involving the rectum and sigmoid . There is also thickening of the cecum. In addition, this could also represent hemorrhage into the bowel wall in the setting of thrombocytopenia. </li></ul></ul>
  13. 13. Repeat Colonoscopy (#3) <ul><li>There were innumerable medium-sized punched-out ulcers in the rectum, rectosigmoid junction, and sigmoid colon. Appeared to have progressed since prior endoscopy. </li></ul><ul><ul><li>Extensive blood, fibrin, and inflammatory cells, with extremely scant epithelial cells. Inadequate tissue to evaluate for amyloid or plasmacytoma. </li></ul></ul>
  14. 14. Repeat EGD/Colonoscopy (#4) <ul><li>EGD normal. </li></ul><ul><li>Colonoscopy showed similar ulceration throughout colon with multiple biopsies taken. </li></ul><ul><ul><li>Ischemic changes with ulceration, colonic epithelium. Congo red stain negative for amyloid. </li></ul></ul>
  15. 15. Differential <ul><li>With multiple unrevealing biopsies, what do you think is still on the differential for colonic ulcers? </li></ul><ul><ul><li>Medication-associated ulcers (Velcade, Acyclovir) </li></ul></ul><ul><ul><li>Ischemic ulcers </li></ul></ul><ul><ul><li>Infectious ulcers </li></ul></ul><ul><ul><li>Amyloid </li></ul></ul><ul><ul><li>Plasmacytoma </li></ul></ul>
  16. 16. Medication-associated colitis <ul><li>“ Acylovir- induced colitis” </li></ul><ul><li>3 cases of hematochezia within 24 hours of starting oral acyclovir (guanosine analog that inhibits viral DNA synthesis) </li></ul><ul><li>No other causes was found, and symptoms resolved with cessation </li></ul><ul><li>Colonoscopy showed friable mucosa with biopsy revealing ulcerated large bowel </li></ul><ul><li>Thought to be caused by local irritation to mucosal surfaces. </li></ul>Wardle TD et al.
  17. 17. Medication-associated colitis <ul><li>Case report of MM patient treated with Velcade (26S proteosome inhibitor) presenting with abdominal pain, hematochezia. </li></ul><ul><li>Colonoscopy showed multiple colonic ulcers, with pathology c/w severe interstitial inflammation. </li></ul><ul><li>Thought to be iatrogenic colitis 2/2 mucositis. </li></ul>Sinischalchi et al.
  18. 18. Intestinal Plasmacytoma <ul><li>Case reports of plasmacytoma in GI tract causing bleeding. </li></ul><ul><li>Extramedullary plasmacytoma account for 4% of plasma cell tumors. </li></ul><ul><li>Most occur in stomach and 20-30% in small intestine. </li></ul><ul><li>Biopsy shows plasma cell infiltrate, reactive for CD138 (plasma cell marker). </li></ul><ul><li>Hypothesis that extramedullary plasmacytomas represent low-grade lymphoma of mucosal lymphoid tissues (MALT) with extensive plasmacytic differentiation. </li></ul>Ammar et al. , Carneiro et al
  19. 19. Clinical course <ul><li>Patient’s bleeding stabilized and he was discharged to home with stable hemoglobin. </li></ul><ul><li>Patient received Revlamid, Dexamethasone, but Velcade was stopped due to possible association with ischemic colitis. </li></ul><ul><li>Re-admitted 1 month later with abdominal pain, diarrhea, hematochezia. Stool studies negative. EGD/ Flex sig was performed. </li></ul>
  20. 20. EGD/Flex Sig (#5) <ul><li>EGD </li></ul><ul><ul><li>Multiple small patchy areas of irregular erosion in the incisura and antrum and on the lesser curvature of the stomach body. </li></ul></ul><ul><ul><li>Normal duodenum. </li></ul></ul><ul><li>Flex sig </li></ul><ul><ul><li>Multiple small ulcers in rectum and circumferential ulcers in sigmoid. Biopsies taken. </li></ul></ul>
  21. 21. Pathology
  22. 22. Diagnosis <ul><li>Amyloidosis of the Colon </li></ul>
  23. 23. Amyloidosis <ul><li>Extracellular deposition of protein fibrils with a β -sheet fibrillar structure. </li></ul><ul><li>Deposits appear homogeneous and amorphous under light microscope, but produce a green birefringence when stained with Congo red and viewed in polarizing microscope. </li></ul>Ebert EC et al. <ul><li>Types of Amyloid: </li></ul><ul><ul><li>Primary or light-chain associated (AL)- 15% have MM </li></ul></ul><ul><ul><li>Secondary or reactive (AA)- chronic inflammatory disorders </li></ul></ul><ul><ul><li>Hemodialysis associated (A β 2M) </li></ul></ul><ul><ul><li>Familial amyloid polyneuropathy (ATTR) </li></ul></ul><ul><ul><li>Senile amyloidosis </li></ul></ul>
  24. 24. Gastrointestinal Amyloid <ul><li>Amyloid may cause GI symptoms extending from mouth to anus </li></ul><ul><li>Amyloid deposition in the GI tract is near universal in systemic AL amyloidosis, but only 30-60% develop GI symptoms. </li></ul><ul><li>When GI tract is involved, frequency of amyloid in biopsy specimens area 100% in the duodenum, 95% in the stomach, 91% in the colorectum, and 72% in the esophagus. Best in blood vessel wall. </li></ul><ul><li>Endoscopically, AL typically forms polypoid protrusions, while AA amyloidosis is characterized by a fine granular appearance </li></ul>Sleisinger and Fordtran., Menke DM et al., Tada S et al., James et al.
  25. 25. GI manifestations <ul><li>Ulcers </li></ul><ul><li>Erosions </li></ul><ul><li>Polypoid lesions </li></ul><ul><li>Submucosal hemorrhage </li></ul><ul><li>Dysphagia </li></ul><ul><li>Gastroparesis </li></ul><ul><li>Constipation, </li></ul><ul><li>Pseudo-obstruction </li></ul><ul><li>Nausea/vomiting </li></ul><ul><li>Weight loss </li></ul><ul><li>Diarrhea </li></ul><ul><li>Steatorrhea </li></ul><ul><li>Protein-losing enteropathy </li></ul>Ebert et al. Amyloid deposition Signs/ symptoms Blood vessel wall Muscle layers Mucosa GI bleeding Dysmotility Malabsorption
  26. 26. Prognosis/ Treatment <ul><li>Median survival for AL amyloidosis less than 2 years, if treated with melphalan and prednisone </li></ul><ul><ul><li>5-year survival improved to 60% with hematopoietic stem cell transplantation </li></ul></ul><ul><li>Treatment of AL involves chemotherapy, while AA involved treatment of underlying disease. </li></ul>
  27. 27. Follow-up Course <ul><li>After amyloid diagnosis, pt given Etoposide, Cyclophosphamide, Dexamethasone. </li></ul><ul><li>2 weeks after EGD/Colonoscopy, pt with fevers, abdominal pain, hypotension. KUB showed no perforation. </li></ul><ul><li>Patient went to the OR. Ex-lap, lysis of adhesions, diverting ileostomy, and blow-hole colostomy was performed, but colectomy deferred due to patient instability. </li></ul><ul><li>Pt with prolonged post-operative hospital course. Re-started on Revlamid. On HD. Just discharged to home. </li></ul>
  28. 28. References <ul><li>Ammar T et al. “Primary Antral Duodenal Extramedullary Plasmacytoma Presenting With Melena” Clinical Gastroenterology and Hepatology. 2010;8:xxxii </li></ul><ul><li>Carneiro FP et al. “Extramedullary plasmocytoma associated with a massive deposit of amyloid in the duodenum.” World J Gastroenterol 2009 July 28; 15(28): 3565-3568 </li></ul><ul><li>Ebert EC et al. “Gastrointestinal Manifestations of Amyloidosis.” Am J Gastroenterol 2008;103:776–787 </li></ul><ul><li>James DG et al. “Clinical Recognition of AL Type Amyloidosis of the Luminal Gastrointestinal Tract” Clinical Gastroenterology and Hepatology. 2007; 5:582–588. </li></ul><ul><li>Maza I et al. “Rectal bleeding as a presenting symptom of AL amyloidosis and multiple myeloma” World J Gastrointest Endosc 2010 January 16; 2(1): 44-46. </li></ul><ul><li>Menke DM et al. “Symptomatic gastric amyloidosis in patients with primary systemic amyloidosis.” Mayo Clin Proc 1993 Aug; 68(8):763-7. </li></ul><ul><li>Sinischalchi A et al. “Bortezomib-related colon mucositis in a multiple myeloma patient” Support Care Cancer (2009) 17:325–327. </li></ul><ul><li>Sleisenger and Fordtran’s “Gastrointestinal and Liver Disease” Ninth ed, 2010. </li></ul><ul><li>Tada S et al. “Endoscopic and biopsy findings of the upper digestive tract in patients with amyloidosis.” Gastrointest Endosc 1990 Jan-Feb;36(1):10-4. </li></ul><ul><li>Wardle TD et al. “Acyclovir-induced colitis” Aliment Pharmacol Ther. 1997 (11): 415-417 </li></ul>

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