Sea-blue histiocytosis in bone marrow in a home TPN patient

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associated fall in HBsAg titres. Interestingly in 11 of these 21 patients seroconversion occurred 4 months before the fall in ALT levels to normal, while in 10 patients, there was a concurrent normalization of ALT with clearance in HBV markers. In contrast, the remaining 9 patients did remain HBeAg positive with associated elevation in ALT levels indicating the continuing liver disease. 7 of these patients remained positive for HBV DNA also. This data suggests that many patients with chronic hepatitis B infection eventually have spontaneous remission. The clinical and bio- chemical remission of active liver disease usually is accompanied by the disappearance of HBeAg and HBV DNA. 527 SEA-BLUE HISTIOCYTOSIS IN BONE MARROW IN A HOME TPN PATIENT Dhanasekaran Ramasamy, M.D., Douglas L. Seidner, M.D., FACG*, Ezra Steiger, M.D., Mohamad Hussein, M.D. Cleveland Clinic, Cleveland, OH. Introduction: Sea-Blue histiocytosis is a morphological finding that can be associated with both acquired conditions of increased cellular turnover and inborn errors of lipid metabolism. The sea-blue histiocyte is a macrophage containing empty vacuoles representing lysosomal bound lipids and dis- tinct pigmented granules stained blue with giemsa solution. The underlying factor is the accumulation of unsaturated lipids either due to increased production, release or to a failure of catabolism. Case Report: A 40 year-old African-american male with a history of suicide attempt due to lye injestion, 10 years ago, with subsequent esopha- gogastrectomy, was admitted with high fevers and chills of one week duration. He was on tube feedings for several years and was placed on TPN due to tube feeding intolerance 10 months prior to admission. Upon admission to the hospital, pan cultures including hickman catheter brush- ing, fungal cultures were obtained and broad spectrum antibiotics were started. The Hickman catheter was removed. All cultures and extensive virological studies were negative. Labs revealed CBC 2.9, Hb of 6.9, Hct 19 and platelets of 55. A bone marrow biopsy showed hypocellularity with Sea-blue histiocytes. The patient was restarted on TPN without lipids and his labs dramatically improved. Repeat bone marrow biopsy after 6 months of lipid free TPN showed increased cellularity. Discussion: Long term TPN is usually given daily at night in a cyclic fashion as intravenous infusion of nutritive mixtures. The syndrome of sea-blue histiocytosis, during TPN, is characterized by bleeding tendency, jaundice, thrombocytopenia, and hepatosplenomegaly, all of these were present in our patient excepy splenomegaly. It is postulated that this histiocytosis is associated with the long term intravenous use of lipid emulsions, due to abnormal metabolism of intravenous long chain fatty acids, leading to abnormal deposits, especially within the reticulo-endo- thelial system. During TPN with lipids, the increased blood lipid levels may affect macrophages leading to excessive cytoplasmic loading of lipids within the histiocytes, and their subsequent incomplete degradation is responsible for the formation of lipopigments. Also, increased blood lipid levels may lead to membrane abnormalities of circulating hematopoietic cells, which could be recognized by intravascular macrophages and elim- inated explaining the hematological abnormalities. 528 SMALL BOWEL DIVERTICULOSIS UNCOMMON CAUSE OF CHRONIC INTERMITTENT ABDOMINAL PAIN Richard Ma, M.D., Richard Prudencio, M.D., Jose Baez, D.O., Manish Tandon, M.D., Julio Ayala, M.D.* Cambridge Health Alliance/ Harvard Medical School, Cambridge, MA. Small-bowel diverticulosis is a rare entity that is usually discovered inci- dentally on radiographic studies or endoscopy. Jejunal diverticulosis occurs in 0.07–2% of the population. Symptoms may occur in 10-40% of the cases. Small-bowel diverticula can be complicated by obstruction, hemor- rhage, peritonitis, pain and bacterial overgrowth. Chronic symptoms of intermittent abdominal pain with flatulence and anemia could occur in 30% of the patients. To diagnose jejunal diverticulosis, enteroclysis is the prefered thecnique. Surgical resection of the involved segment must be done in patients with symptoms or complications. The patient is a 75 year-old male who presented with a severe diffuse abdominal pain. He had a history of chronic intermittent abdominal pain, gastro-esophageal reflux disease, and left sided diverticulosis diagnosed on colonoscopy, hyperten- sion, benign prostatic hypertrohy, prostatitis. The abdominal pain was intermittent and had been increasing in intensity. An Abdominal CT scan done with oral contrast showed colonic diverticula but no sign of inflam- mation. CBC, Chem 7, and liver tests were within normal limits. An upper GI series with small bowel series follow through showed multiple jujunal diverticula. The patient’s symptoms resolved with 14 days of antibiotic therapy (levofloxacine 400 mg PO QD). 529 CYTOMEGALOVIRUS COLITIS: A RARE CAUSE OF MASSIVE LOWER GI BLEED IN AN IMMUNOCOMPETENT PATIENT Anand Madan, M.D., Niti Madan, M.D., John O’Brien, M.D.* Southern Illinois University School of Medicine, Springfield, IL. A 70 year-old white female was admitted to our institution for right hip disarticulation due to severe peripheral vascular disease. Her past medical history was significant for Hypertension, GERD, Coronary artery disease and peripheral vascular disease. She was on long term aspirin therapy. After the hip surgery, patient was placed on Warfarin. On post-operative day 6, patient developed massive lower gastrointestinal bleeding with passage of bright red blood per rectum. This was assoociated with severe hypotension with systolic blood pressue around 70 mm Hg. Laboratoty data showed hemoglobin of 7.9 gm%, a drop of about 4 grams since admission. Patient also had INR of 3.8 which was reversed with Vitamin K injections and fresh frozen plasma infusions. Even correction of coagulopathy did not result in slowing of lower GI bleeding. An emergent Tc-99m labelled RBC scan was performed which showed increased localization of tracer in the rectum, this was followed by an angiogram which failed to reveal the site of bleeding. An emergent surgery (left hemicolectomy/rectosigmoid resec- tion) was considered due to continued bleeding but patient declined to undergo any surgical intervention. After hemodynamic stabalization, colonoscopy was performed which showed numerous discrete ulcers scat- tered throughout the colon including rectum. Biopsies obtained from these ulcers showed areas of ulceration with acute inflammation and granulation tissue formation. Focally, within the lamina propria, there were occassional cells with marked cytomegaly and smudged chromatin with some intracy- toplasmic viral inclusions. The tissue was negative immunohistochemically for antigens of herpes simplex virus type 1 and type 2. An immunohisto- chemical stain for cytomegalovirus antigen shows numerous immunohis- tochemically positive cells within the lamina propria, both within areas of ulceration and in some areas of otherwise unremarkable mucosa. Patient was subsequently treated with Gancyclovir with resolution of her symp- toms. Our patient did not have any other underlying immunocompromising illness which could have predisposed her to CMV colitis. Our case demon- states the importance of considering rare infectious causes of GI bleed in immunocompetent persons which could otherwise prove fatal. 530 AN OVERLOOKED CAUSE OF IRON DEFICIENCY ANEMIA Rajesh Sharma, M.D.*, Paul J. Ruh, M.D. Aurora Medical Center, Two Rivers, WI. Iron deficiency anemia (IDA) in elderly is presumed to be related to chronic GI blood loss or sometimes related to poor absorption from achlorhydria or celiac disease. Certain causes of iron loss may not be actively sought after on history. A 69 year-old white female was seen by her PCP with c/o tiredness and dyspnea on exertion. CBC showed Hb 11.7 gm, HCT 36.9 with MCV 78.9. Serum iron was 17 ug/dl (37–170) with ferritin of 13 ng/ml. Last CBC on the chart two years ago showed Hb 12.8, MCV 89.6. S178 Abstracts AJG – Vol. 98, No. 9, Suppl., 2003

Transcript of Sea-blue histiocytosis in bone marrow in a home TPN patient

Page 1: Sea-blue histiocytosis in bone marrow in a home TPN patient

associated fall in HBsAg titres. Interestingly in 11 of these 21 patientsseroconversion occurred 4 months before the fall in ALT levels to normal,while in 10 patients, there was a concurrent normalization of ALT withclearance in HBV markers. In contrast, the remaining 9 patients did remainHBeAg positive with associated elevation in ALT levels indicating thecontinuing liver disease. 7 of these patients remained positive for HBVDNA also. This data suggests that many patients with chronic hepatitis Binfection eventually have spontaneous remission. The clinical and bio-chemical remission of active liver disease usually is accompanied by thedisappearance of HBeAg and HBV DNA.

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SEA-BLUE HISTIOCYTOSIS IN BONE MARROW IN A HOMETPN PATIENTDhanasekaran Ramasamy, M.D., Douglas L. Seidner, M.D., FACG*,Ezra Steiger, M.D., Mohamad Hussein, M.D. Cleveland Clinic,Cleveland, OH.

Introduction: Sea-Blue histiocytosis is a morphological finding that can beassociated with both acquired conditions of increased cellular turnover andinborn errors of lipid metabolism. The sea-blue histiocyte is a macrophagecontaining empty vacuoles representing lysosomal bound lipids and dis-tinct pigmented granules stained blue with giemsa solution. The underlyingfactor is the accumulation of unsaturated lipids either due to increasedproduction, release or to a failure of catabolism.Case Report: A 40 year-old African-american male with a history ofsuicide attempt due to lye injestion, 10 years ago, with subsequent esopha-gogastrectomy, was admitted with high fevers and chills of one weekduration. He was on tube feedings for several years and was placed on TPNdue to tube feeding intolerance 10 months prior to admission. Uponadmission to the hospital, pan cultures including hickman catheter brush-ing, fungal cultures were obtained and broad spectrum antibiotics werestarted. The Hickman catheter was removed. All cultures and extensivevirological studies were negative. Labs revealed CBC 2.9, Hb of 6.9, Hct19 and platelets of 55. A bone marrow biopsy showed hypocellularity withSea-blue histiocytes. The patient was restarted on TPN without lipids andhis labs dramatically improved. Repeat bone marrow biopsy after 6 monthsof lipid free TPN showed increased cellularity.Discussion: Long term TPN is usually given daily at night in a cyclicfashion as intravenous infusion of nutritive mixtures. The syndrome ofsea-blue histiocytosis, during TPN, is characterized by bleeding tendency,jaundice, thrombocytopenia, and hepatosplenomegaly, all of these werepresent in our patient excepy splenomegaly. It is postulated that thishistiocytosis is associated with the long term intravenous use of lipidemulsions, due to abnormal metabolism of intravenous long chain fattyacids, leading to abnormal deposits, especially within the reticulo-endo-thelial system. During TPN with lipids, the increased blood lipid levels mayaffect macrophages leading to excessive cytoplasmic loading of lipidswithin the histiocytes, and their subsequent incomplete degradation isresponsible for the formation of lipopigments. Also, increased blood lipidlevels may lead to membrane abnormalities of circulating hematopoieticcells, which could be recognized by intravascular macrophages and elim-inated explaining the hematological abnormalities.

528

SMALL BOWEL DIVERTICULOSIS UNCOMMON CAUSE OFCHRONIC INTERMITTENT ABDOMINAL PAINRichard Ma, M.D., Richard Prudencio, M.D., Jose Baez, D.O.,Manish Tandon, M.D., Julio Ayala, M.D.* Cambridge Health Alliance/Harvard Medical School, Cambridge, MA.

Small-bowel diverticulosis is a rare entity that is usually discovered inci-dentally on radiographic studies or endoscopy. Jejunal diverticulosis occursin 0.07–2% of the population. Symptoms may occur in 10-40% of thecases. Small-bowel diverticula can be complicated by obstruction, hemor-rhage, peritonitis, pain and bacterial overgrowth. Chronic symptoms of

intermittent abdominal pain with flatulence and anemia could occur in 30%of the patients. To diagnose jejunal diverticulosis, enteroclysis is theprefered thecnique. Surgical resection of the involved segment must bedone in patients with symptoms or complications. The patient is a 75year-old male who presented with a severe diffuse abdominal pain. He hada history of chronic intermittent abdominal pain, gastro-esophageal refluxdisease, and left sided diverticulosis diagnosed on colonoscopy, hyperten-sion, benign prostatic hypertrohy, prostatitis. The abdominal pain wasintermittent and had been increasing in intensity. An Abdominal CT scandone with oral contrast showed colonic diverticula but no sign of inflam-mation. CBC, Chem 7, and liver tests were within normal limits. An upperGI series with small bowel series follow through showed multiple jujunaldiverticula. The patient’s symptoms resolved with 14 days of antibiotictherapy (levofloxacine 400 mg PO QD).

529

CYTOMEGALOVIRUS COLITIS: A RARE CAUSE OF MASSIVELOWER GI BLEED IN AN IMMUNOCOMPETENT PATIENTAnand Madan, M.D., Niti Madan, M.D., John O’Brien, M.D.* SouthernIllinois University School of Medicine, Springfield, IL.

A 70 year-old white female was admitted to our institution for right hipdisarticulation due to severe peripheral vascular disease. Her past medicalhistory was significant for Hypertension, GERD, Coronary artery diseaseand peripheral vascular disease. She was on long term aspirin therapy.After the hip surgery, patient was placed on Warfarin. On post-operativeday 6, patient developed massive lower gastrointestinal bleeding withpassage of bright red blood per rectum. This was assoociated with severehypotension with systolic blood pressue around 70 mm Hg. Laboratoty datashowed hemoglobin of 7.9 gm%, a drop of about 4 grams since admission.Patient also had INR of 3.8 which was reversed with Vitamin K injectionsand fresh frozen plasma infusions. Even correction of coagulopathy did notresult in slowing of lower GI bleeding. An emergent Tc-99m labelled RBCscan was performed which showed increased localization of tracer in therectum, this was followed by an angiogram which failed to reveal the siteof bleeding. An emergent surgery (left hemicolectomy/rectosigmoid resec-tion) was considered due to continued bleeding but patient declined toundergo any surgical intervention. After hemodynamic stabalization,colonoscopy was performed which showed numerous discrete ulcers scat-tered throughout the colon including rectum. Biopsies obtained from theseulcers showed areas of ulceration with acute inflammation and granulationtissue formation. Focally, within the lamina propria, there were occassionalcells with marked cytomegaly and smudged chromatin with some intracy-toplasmic viral inclusions. The tissue was negative immunohistochemicallyfor antigens of herpes simplex virus type 1 and type 2. An immunohisto-chemical stain for cytomegalovirus antigen shows numerous immunohis-tochemically positive cells within the lamina propria, both within areas ofulceration and in some areas of otherwise unremarkable mucosa. Patientwas subsequently treated with Gancyclovir with resolution of her symp-toms. Our patient did not have any other underlying immunocompromisingillness which could have predisposed her to CMV colitis. Our case demon-states the importance of considering rare infectious causes of GI bleed inimmunocompetent persons which could otherwise prove fatal.

530

AN OVERLOOKED CAUSE OF IRON DEFICIENCY ANEMIARajesh Sharma, M.D.*, Paul J. Ruh, M.D. Aurora Medical Center, TwoRivers, WI.

Iron deficiency anemia (IDA) in elderly is presumed to be related to chronicGI blood loss or sometimes related to poor absorption from achlorhydria orceliac disease. Certain causes of iron loss may not be actively sought afteron history. A 69 year-old white female was seen by her PCP with c/otiredness and dyspnea on exertion. CBC showed Hb 11.7 gm, HCT 36.9with MCV 78.9. Serum iron was 17 ug/dl (37–170) with ferritin of 13ng/ml. Last CBC on the chart two years ago showed Hb 12.8, MCV 89.6.

S178 Abstracts AJG – Vol. 98, No. 9, Suppl., 2003