Profound Iron Deficiency Anemia Research Articles

A236 ENDOSCOPIC MANAGEMENT OF RARE JEJUNAL SOLITARY PEUTZ-JEGHERS-TYPE POLYP IN AN ELDERLY PATIENT WITHOUT A POLYPOSIS SYNDROME: A CASE REPORT AND REVIEW OF THE LITERATURE

Abstract Background Solitary Peutz-Jeghers-type polyp (SPJP) is a rare hamartomatous lesion. It is considered as a different entity from Peutz-Jeghers syndrome despite similar histopathological findings. It can be found in the GI tract but rarely in the jejunum. Jejunal SPJP are susceptible to necrosis, ulceration, and intussusception, resulting in GI bleeding or small bowel obstruction. Purpose We describe a case of subacute gastrointestinal bleeding secondary to jejunal SPJP to share our approach to this challenging case using therapeutic endoscopy Method An 81-year-old male patient with a history of atrial fibrillation on warfarin with stable therapeutic INR levels, who presented with 1-week history of melena, generalized fatigue and shortness of breath on exertion and was found to have profound iron deficiency anemia. Esophageal gastroduodenoscopy and colonoscopy failed to identify the source of bleeding; however, double balloon enteroscopy detected a 4 cm polyp with stalk in the proximal jejunum. Endoscopic polypectomy was performed and the whole polyp was removed. Histopathological examination was consistent with Peutz-Jeghers polyp. The oropharyngeal and skin examination was negative for mucocutaneous hyperpigmented macules and the genetic analysis was negative for STK11 mutation. Follow up magnetic resonance enterography and video capsule endoscopy did not reveal any other polypoid lesion in the GI tract. The patient’s symptoms resolved gradually, and his hemoglobin level returned back to normal levels within 6 months. Result(s) To our knowledge this is the first case of endoscopic polypectomy during balloon-assisted enteroscopy for jejunal SPJP. Conclusion(s) Conclusion Solitary Peutz-Jeghers hamartomas are a rare occurrence but should be part of the differential diagnosis for abdominal pain from intussusception, or small bowel bleeding even in elderly patient. Balloon assisted enteroscopy is a valuable technique for the management of solitary small bowel lesions. Please acknowledge all funding agencies by checking the applicable boxes below None Disclosure of Interest None Declared LIVER AND BILIARY DISEASE

32700 Association between body lice infestation and decreased hemoglobin values

Background: Pediculus humanus humanus (body lice) survive on blood meals taken from human hosts. Case reports have described profound iron deficiency anemia associated with extensive body lice infestation, but larger epidemiologic studies have not been performed.

Concurrent cerebral arterial and venous sinus thrombosis revealing celiac disease- a case report and literature review

BackgroundCeliac disease is an autoimmune condition characterized by an inappropriate immune reaction against gluten. It classically presents as chronic diarrhea, bloating, and nausea in addition to malabsorption symptoms such as weight loss and micronutrient deficiency. We report the first case of coinciding cerebral infarction and venous sinus thrombosis unveiling the diagnosis of celiac disease.Case presentationA 40-year old female patient with a four-day history of severe diarrhea presented with right hemiplegia and altered mental status. Imaging revealed left middle cerebral artery occlusion and left transverse and sigmoid venous sinus thrombosis, along with left jugular vein thrombosis. Her laboratory evaluation was notable for profound iron deficiency anemia, thrombocytosis, and hyperhomocysteinemia. Her positive anti-tissue transglutaminase IgA antibodies and ensuing duodenal biopsy confirmed the diagnosis of celiac disease.ConclusionsCeliac disease has a wide range of intestinal and extraintestinal manifestations and can present with thrombotic events in young patients with iron deficiency and hyperhomocysteinemia.

Mucosal miR-3677 is over-expressed in cirrhotic patients with gastric antral vascular ectasia (GAVE)

Introduction: Gastric antral vascular ectasia (GAVE) is a rare vasculopathy that associates several diseases, most commonly liver cirrhosis. It usually presents as an occult gastrointestinal bleeding leading to profound iron deficiency anemia. We hypothesized that GAVE is local mucosal pathology dependent on genetic mechanisms, and the purpose of the study was to characterize miRNAs expression in gastric tissue of patients with cirrhosis and GAVE.Materials and methods: Thirteen patients with GAVE and cirrhosis and 35 healthy subjects were recruited. Microarray analysis and comparative microRNA study was done by quantitative polymerase chain reaction (qPCR). The microarray scores were grouped with use of the hierarchical clusterization analysis and miRNA target prediction was done with TargetScan 6.2 algorithm and Gene Ontology analysis (DIANA-miRPath).Results: Concentration of miR-3677 in GAVE-affected mucosa was higher by 72% in comparison with GAVE-free mucosa of patients with cirrhosis (33.7 vs. 35.6 PCR cycles; p < .001) and by 45% in comparison with normal mucosa (33.7 vs. 34.9 PCR cycles; p < .05). According to Gene Ontology analysis miR-3677 was related to angiopoietin-like protein 4 (ANGPTL4) gene.Conclusion: GAVE in liver cirrhosis is associated with increased expression of miR-3667 that may be linked with ANGPTL4 gene.

Systemic Bevacizumab for the Treatment of Chronic Bleeding in Hereditary Hemorrhagic Telangiectasia

▪Introduction: Hereditary hemorrhagic telangiectasia (HHT) is a rare hereditary multisystem vascular disorder causing visceral arteriovenous malformations (AVMs) and mucocutaneous bleeding. Chronic gastrointestinal bleeding and epistaxis frequently produce profound iron deficiency anemia refractory to conventional treatment. Patients with HHT have elevations in vascular endothelial growth factor (VEGF), so the anti-VEGF agent bevacizumab, a recombinant, humanized monoclonal IgG1 antibody that binds to and neutralizes circulating VEGF, is a promising systemic HHT therapy. Currently, data pertaining to the efficacy of bevacizumab for the treatment of HHT is limited to case reports and retrospective studies that do not describe the effect of bevacizumab on objective hematologic parameters (Guilhem et al 2017, Iyer et al 2018). As our institution is an HHT center that has developed a pathway for the use of bevacizumab in HHT patients, we performed a retrospective analysis assessing the efficacy of bevacizumab to alleviate chronic bleeding as measured by improvement in hemoglobin concentration, need for red cell transfusions and iron infusions, and epistaxis control. Management of bleeding in HHT patients is an off-label use of bevacizumab.Methods: All HHT patients treated with systemic bevacizumab for chronic bleeding were selected for retrospective analysis. Data was collected for each patient over a 14-month course, divided into a pretreatment period (six months), induction period (two months), and maintenance period (six months) and included demographics, baseline HHT characteristics, epistaxis grade, surgical interventions, bevacizumab dosing, adverse events, hemoglobin, red cell transfusions, intravenous iron infusions, and other anemia and/or bleeding-directed therapies. Additionally, the peak hemoglobin on bevacizumab over the course of all available follow-up was collected for each patient. Our institution's bevacizumab treatment pathway began with an induction phase (5 mg/kg of IV bevacizumab every two weeks for four treatments) followed by a maintenance phase (5 mg/kg administered monthly thereafter). Statistical tests used in data analysis included the paired t-test, Wilcoxon signed rank test, and Fisher's exact test.Results: 13 HHT patients were treated with bevacizumab for chronic bleeding for a median of 13.9 (range, 4.9-30.1) months. Baseline patient characteristics are shown in Table 1 and hematologic parameters during the pretreatment, induction, and maintenance periods are shown in Table 2. Compared with pretreatment values, bevacizumab treatment increased the mean hemoglobin by 4.0 g/dL (95% CI, 2.6-5.3 g/dL) [mean (95% CI) hemoglobin 8.5 (7.8, 9.9) g/dL versus 12.5 (11.2, 13.7) g/dL, p<0.001)], reduced red cell units transfused by 92% [median of 6 (range, 0-59) units versus 0 (range, 0-15) units, p=0.004], and reduced quantity of iron infused by 73% [mean (95% CI) 462 (257, 668) mg/month versus 126 (75, 178) mg/month, p=0.002]. The peak hemoglobin value measured over the course of all available follow up ranged from 12.1-17.6 mg/dL (Figure 1), with all but two patients (85%) achieving a hemoglobin within the normal range for gender. Twelve of 13 patients did not require red cell transfusions during the entire maintenance period and 11 of 13 patients did not require iron infusions during the latter half of the maintenance period (Figure 2). Epistaxis control (reduction of epistaxis grade to <2) was achieved in 85% with bevacizumab, versus 0% before treatment (p<0.001) and all but one patient experienced improvement in epistaxis grade. No patient required nasal or GI procedures during the maintenance period. Four out of 5 patients receiving antifibrinolytic agents or erythropoiesis-stimulating agents prior to initiation of bevacizumab were able to discontinue these agents during bevacizumab induction and not resume them thereafter. Bevacizumab was well-tolerated, with two patients (15%) developing grade 3 hypertension requiring medical management.Conclusions: Systemic bevacizumab was highly effective to treat chronic bleeding and iron deficiency anemia in HHT. This study is the first to demonstrate the impact of bevacizumab on objective hematologic parameters, such as hemoglobin and iron infusion requirements. Further study is needed to confirm the benefit magnitude and define optimal dosing, treatment duration, and long-term safety. DisclosuresAl-Samkari:Agios: Consultancy. Kuter:Protalex: Research Funding; Rigel: Consultancy, Research Funding; Novartis: Consultancy; Amgen Inc.: Consultancy; Argenx: Consultancy; Bioverativ: Consultancy, Research Funding; BMS: Research Funding; Syntimmune: Consultancy; Pfizer: Consultancy; Dova Pharmaceuticals: Consultancy, Membership on an entity's Board of Directors or advisory committees; Principia: Research Funding; ONO: Consultancy.

An Unexpected Cause of Profound Iron Deficiency Anemia

Introduction: Solitary fibrous tumors (SFTs) are rare spindle cell tumors that usually arise from the pleura. In a few case reports, they have been found in other sites. We present a rare case of gastric SFT presenting with bloody emesis. A 50-year-old female presented with coffee ground emesis associated with melena for 6 days. Physical examination was significant for pallor and tachycardia. Abdominal examination was benign. Laboratory results revealed iron deficiency anemia with a hemoglobin of 1.8 but otherwise unremarkable. EGD showed a 4-5 cm bilobed mass near the junction of the stomach antrum and body. Biopsy was inconclusive. Radiographic staging confirmed gastric mass without evidence of metastasis. Laparotomy with wedge resection of the stomach showed a 5.0 x 3.8 x 3.1 cm, exophytic mass with central ulceration. Pathology showed gastric wall containing spindle cell tumor ulcerating through the mucosa. Histologic and immunohistochemistry picture (diffuse CD34 and bcl2 positivity) fit the diagnosis of angiofibromatous variant of SFT with insufficient histological criteria for malignancy. Klemperer first described SFTs in 1931. Only 15-20% are malignant. The median age of occurrence is 45 to 50 years with both sexes equally affected. Presenting symptoms depend on location and size of the tumor. Diagnosis is primarily based on histopathology which is the gold standard as clinical features and imaging play a limited role. The most effective treatment modality is complete surgical resection. To our best knowledge, we present the first case of gastric SFT of the angiofibromatous variant presenting with profound anemia.Figure 1: Gastric bi-lobed mass.Figure 2: Gross pathology of bi-lobed gastric mass.

A Classic Case of Diarrhea in a Severely Immune-compromised Patient

Purpose: A classic presentation of an exceedingly rare entity. Case Report: A 28-year-old African-American female with HIV/AIDS most recent CD4 1, VL 600,000 copies/mL who is non-compliant with HAART therapy presented to the emergency department with complaints of profuse watery non-bloody diarrhea for three weeks associated with abdominal cramping and night sweats. She also had 10-pound unintentional weight loss over the three weeks. She denied nausea and vomiting. She denied sick contacts and recent travel. Her past medical history also includes: oral/esophageal candidiasis, pneumocystis jirovecii pneumonia and molluscum contagiosum. On examination, she was found to have a fever of 103.8F, hypotension and tachycardia. She appeared severely cachectic and listless. Her abdomen was scaphoid with hyperactive but normo-pitched bowel sounds, soft and non-tender. The stool was liquid, brown mixed with mucous; there was no bright red blood or melena. Labs were significant for profound iron deficiency anemia, thrombocytosis and electrolytes disturbance. The liver function tests were within normal limits. Stool studies and culture were negative. Rectal swab viral culture was also negative. CAT scan of the abdomen and pelvis revealed hepatomegaly, splenomegaly with multiple hypoattenuated lesions in the spleen and multiple mesenteric lymph nodes. Flexible- sigmoidoscopy was normal and the pathology on random biopsies showed chronic non-specific inflammation. Esophagogastroduodenoscopy revealed thickening pale nodular duodenal folds interdigitated with atrophic friable mucosa (Figure 1). Pathology was significant for numerous histiocytes within the lamina propria with PAS and AFB stain positive revealing numerous intracellular organisms morphologically consistent with Mycobacterium avium-intracellulare complex. Subsequently, blood cultures returned positive for Mycobacterium avium-intracellular complex as well. She was started on ethambutol, clarithromycin, and rifabutin, and her symptoms resolved.Figure

Extramedullary Plasmacytoma of the Cecum: A Rare Cause of Iron Deficiency Anemia

Introduction: Plasmacytomas are solid myelomatous tumors composed of atypical plasma cells, which can be formed within bone or less commonly within soft tissue. Plasmacytomas are commonly associated with multiple myeloma (MM), however they may manifest outside the context of MM as solitary plasmacytomas. Extramedullary plasmacytomas are most commonly found in the aerodigestive tract, however they may present anywhere in the body. Here we present a rare case of a woman with iron deficiency anemia secondary to a cecal plasmacytoma not associated with MM. Case Report: A 73-year-old woman presented with iron deficiency anemia. Two years prior she had been diagnosed with a solitary extramedullary plasmacytoma in her right posterior mediastinum with intraspinal extension to T3. She underwent curative T2-T5 laminectomy and radiotherapy. Her disease was considered in remission at the time of presentation. After treatment of her profound iron deficiency anemia with intravenous iron, a single contrast barium enema was performed. It showed a large polypoid lesion in the lateral proximal ascending colon below the level of the ileocecal valve. These findings were indicative of colonic neoplasm and she was referred for colonoscopy. Colonoscopy confirmed the presence of a 4 cm fungating tumor at this location. This tumor was not amenable to endoscopic removal. Biopsies were taken for pathology which demonstrated plasmacytic infiltrate with frequent mitotic activity consistent with extramedullary plasmacytomas. Her β2 microglobulin level was elevated (282.5 nmol/L). She was referred back to oncology services where she was treated with concurrent prednisone and radiotherapy - 45 gray in 25 fractions. Discussion: Colonic extramedullary plasmacytomas are exceedingly rare with fewer than 25 cases reported in the literature. Any area of the gastrointestinal tract may be involved; however the most common site of involvement is the sigmoid colon. The most common presenting symptom of colonic plasmacytoma is abdominal pain; other symptoms have also been reported including overt bleeding, large bowel obstruction, and intussusception. We report a rare diagnosis of colonic extramedullary plasmacytoma from a history of iron deficiency anemia without presentation of the aforementioned symptoms. Treatment of plasmacytomas of the colon most commonly involve surgical resection. Radiotherapy and chemotherapy have only played a modest role in treatment. In our case the patient was felt to be a poor surgical candidate, and successful radiotherapy was performed.

Profound Iron Deficiency Anemia in a Patient with Diffuse Cavernous Hemangioma

Purpose: A 39-year-old male presented with dyspnea and recurrent hematochezia. Colonoscopy 10 years prior showed vascular lesions and hemorrhoids. He had no follow up since then. Physical exam revealed axillary lymphadenopathy. Laboratory evaluation revealed severe iron deficiency anemia (Hct 16, MCV 52, Iron 12). CT revealed a right hilar mass compressing the right mainstem bronchus and multiple lung nodules. Rectum was diffusely thickened extending into the sigmoid colon, associated with numerous phleboliths. (Figure 1) Several hepatic lesions were seen. Colonoscopy showed increased mucosal vascular pattern from the rectum to distal sigmoid (Figure 2) and a patchy area in transverse colon. Endoscopic ultrasound demonstrated edematous vascular structures in this distribution. A heterogeneous, hypoechoic, circumferential lesion measuring 18 mm in maximal thickness involving all layers was noted. Doppler ultrasound showed vascular flow in the lesion (Figure 3). Calcified phleboliths were seen in the rectal wall. The patient was diagnosed with cavernous hemangiomatosis of the colon and pulmonary non-small cell carcinoma. Colonic cavernous hemangiomatosis is rare and benign, with infrequent simultaneous liver involvement. There is frequent delay in diagnosis. Surgical resection is the preferred treatment modality for diffuse disease.Figure 1: No Caption available.Figure: [1178] Figure 2Figure: [1178] Figure 3

Drug-Induced Idiopathic Portal Hypertension Mimicking “Cryptogenic” Cirrhosis in a HIV Patient

Introduction: Drug-induced idiopathic portal hypertension (IPH), which may mimic complications of “cryptogenic” cirrhosis, is an exceedingly rare complication of antiretroviral therapy (ART). Case: A 50-year-old Caucasian man with a 20-year history of stable human immunodeficiency virus (HIV) infection on ART therapy presented with fatigue, new onset ascites, and profound iron-deficiency anemia. Endoscopies revealed only portal congestive gastopathy and grade 3 esopahgeal varices. Esphageal varices were banded and a non-selective beta-blocker and diuretics initiated. Computerized tomography scan of the abdomen and revealed only large amount of ascites, hepatosplenomegaly and patent hepatic and portal veins. Liver biopsy revealed mild chronic hepatitis with pericellular and periportal fibrosis (stage 2 of 4). Patient was referred to our center for management of “cryptogenic” cirrhosis. Medical history was positive only for HIV, hypertension and hyperlipidemia. He denied alcohol consumption, smoking, or family history of liver disease. A comprehensive serologic evaluation for alternative causes of chronic liver disease and diagnostic studies for cardiopulmonary disease were negative. His ART included tenofovir/emtricitabine, didanosine, and darunavir with ritonavir boosting. On exam, he appeared chronically ill, muscle wasted, had marked ascites and mild hepatosplenomegaly. Laboratory studies revealed an albumin 2.2 g/dl, AST 83 U/L, ALT 60 U/L, iron-deficiency anemia, CD4 count >250 cells, and undetectable HIV viral load. Calculated MELD score was 8. Ascites fluid analysis revealed a serum to ascites albumin gradient of >1.1, protein <3.0 g/dl, <250 white blood cells, and negative cytology. Despite dietary sodium restriction and increasing diuretic doses, he required routine large-volume paracenteses and supportive blood transfusions. Mean portal pressure was 17 mmHg. Transjugular intrahepatic portosystemic shunt was placed with improvement, but not resolution, of ascites or anemia. Due to transient increase in HIV viral load, didanosine, darunavir, and ritonavir were discontinued and raltegravir added to tenofovir/emtricitabine. Within 3 months, all complications of portal hypertension resolved. Liver biopsy 2 years after his initial presentation confirmed normal liver histology with no hepatic fibrosis. Conclusion: Drug-induced IPH is a very rare complication of didanosine, either as monotherapy or in combination with other ART drugs. Timely recognition of ART-related IPH is essential to needless diagnostic/herapeutic interventions for portal hypertension and “cryptogenic” cirrhosis as IPH will resolve with discontinuation of the culprit drug.

Nutmeg liver and ascites resulting from a membranous web obstruction at the hepatic origin of the inferior vena cava

A 30-year-old female presented to the hospital for an evaluation of anemia and intermittent abdominal distention. Laboratory studies revealed a profound iron-deficiency anemia with a hemoglobin level of 6.8, mean cell volume of 62, and a ferritin value of 3. BCS, Budd-Chiari syndrome; CAT, computerized axial tomography; IR, interventional radiology; IVC, inferior vena cava; PH, portal hypertension; US, ultrasound. On physical exam, her temperature was 97.9°F, pulse was 82, respiratory rate was 17, and blood pressure was 94/70. She was found to have hepatomegaly and moderate ascites on abdominal exam and was heme negative on rectal exam. An esophagogastroduodenoscopy and colonoscopy were unremarkable for a source of blood loss, and small bowel biopsies were not consistent with celiac sprue. A right upper quadrant ultrasound (US) revealed moderate ascites and hepatosplenomegaly. The liver had a lobular contour to it with increased echogenicity. A subsequent computerized axial tomography (CAT) scan illustrated a “nutmeg liver” and ascites (Fig. 1). A repeat US with Doppler showed patent hepatic and portal veins with normal direction of flow. Her liver function studies were all within normal limits, along with hepatitis serologies, antinuclear antibody, and Epstein-Barr virus titers. The ascites was sampled, revealing a serum ascites albumin gradient of >1.1 and a protein level of <2.5, consistent with portal hypertension (PH). Hepatic venous pressures were attempted by interventional radiology (IR). The pressure in the intrahepatic portion of the inferior vena cava (IVC) was elevated to 14 mmHg, and the right heart pressure was normal at 4 mmHg, yielding a 10-mmHg venous pressure gradient between the supra- and intrahepatic portion of the IVC. Attempts were made to cannulate the hepatic veins to obtain free hepatic pressures; however, these were unsuccessful because of a narrowing in the intrahepatic portion of the IVC. This prompted a third US in IR, demonstrating a web and turbulent flow at the origin of the hepatic vein from the IVC. IR performed a transhepatic venogram, and the web was found at the confluence of the middle hepatic vein and the IVC. The initial pressure gradient between the middle hepatic vein and the right atrium was significantly elevated at 20 mmHg. IR executed a successful angioplasty of the web with a 12 mm × 4 cm balloon, and the pressure gradient dropped to 4 mmHg (Fig. 2). The patient recovered nicely, with resolution of her ascites and symptoms. A 1-month follow-up US illustrated no residual stenosis (Fig. 3). A CAT scan 5 months later showed a dramatic improvement in the appearance of her liver (Fig. 4). As for her anemia, it was believed to be secondary to her menses and corrected nicely with iron supplementation. Budd-Chiari syndrome (BCS) occurs as a result of PH from hepatic venous outflow obstruction, usually from a hepatic vein thrombosis. Membranous webs can also rarely form in the hepatic venous system, eliciting the same organic response. Venous thrombosis can require pharmacologic thrombolysis and even liver transplantation. However, BCS resulting from membranous web obstruction can be successfully treated with angioplasty and, sometimes, with stenting.

Nutmeg Liver and Ascites Resulting from a Membranous Web Obstruction at the Hepatic Origin of the Inferior Vena Cava

Purpose: A 30 year old female presented to the hospital for evaluation of anemia found on outpatient blood work along with complaints of intermittent abdominal distention. Laboratory studies revealed a profound iron deficiency anemia with a hemoglobin of 6.8, MCV of 62, and a ferritin of 3. On physical exam, she was found to have hepatomegaly and moderate ascites. She was heme negative on rectal exam. An EGD and colonoscopy were unremarkable for a source of blood loss, and small bowel biopsies were not consistent with celiac sprue. To further evaluate her liver, an ultrasound was performed, revealing moderate ascites and hepatosplenomegaly. The liver had a lobular contour to it with increased echogenicity. A subsequent CAT scan of the abdomen and pelvis illustrated a “nutmeg liver” and a large amount of ascites.(Fig 1) A repeat ultrasound with Doppler showed patent hepatic and portal veins with normal direction of flow. Her liver function studies were all within normal limits and hepatitis serologies, ANA, and EBV titers were all negative. The ascitic fluid was sampled with a diagnostic paracentesis. The fluid revealed a SAAG of >1.1 and a protein of <2.5, consistent with portal hypertension. Hepatic venous pressures were performed by interventional radiology (IR) to help determine the etiology of the patient's liver disease. The intrahepatic pressure was elevated to 14 mmHg and the right heart pressure was normal at 4 mmHg; yielding a 10 mmHg venous pressure gradient between the infra- and intrahepatic portion of the inferior vena cava (IVC). This prompted a third ultrasound in IR, demonstrating a web and turbulent flow at the origin of the hepatic veins from the IVC. A few days later, IR executed a successful angioplasty of the web with a 12 mm x 4 cm balloon. (Fig 2) The pressure gradient dropped to 4 mmHg following the breaking of the web. The patient recovered nicely, with resolution of her ascites and symptomatic improvement. She underwent a follow up ultrasound 1 month after her procedure that illustrated no significant residual stenosis and a decrease in hepatomegaly.Figure 1Figure 2

Severe Iron Deficiency Anemia and Lice Infestation

Background Lice infestation is a commonly encountered disorder in emergency medicine. The louse survives from a blood meal from its host; hence, iron deficiency anemia is a theoretic possibility. A limited number of reports of severe iron deficiency anemia have appeared in the veterinary literature, but a thorough review of the medical literature did not reveal a single instance in human beings. Objective We report a small case series of patients with heavy louse infestation and profound iron deficiency anemia. Case report The index case along with two other cases discovered from an exhaustive search of 4 years of the institution’s Emergency Department records all had heavy infestation with head and body lice. Laboratory evaluation revealed serum hemoglobin levels under 6 gm/dL, low serum ferritin levels, and microcytic red blood cell indices. All patients were admitted to the hospital, received transfusions, and had evaluation of their anemia. No patient had evidence of gastrointestinal blood loss or alternative explanation for their anemia. Conclusions Although cause and effect cannot be established from this case series, to the best of our knowledge, this is the first published evidence of a provocative association of louse infestation and severe iron deficiency anemia in humans.

Endoscopically Diagnosed Blue Rubber Bleb Nevus Syndrome in an Elderly Patient with Anemia and Heme Positive Stool

Purpose: Blue Rubber Bleb Nevus syndrome (BRBNS) is a rare disorder that is characterized by multiple recurrent vascular malformations such as hemangiomas, and these primarily involve the skin and the gastrointestinal tract. Typically, it results in profound iron deficiency anemia or overt gastrointestinal blood loss due to involvement of the internal organs. A 79 year-old Caucasian man with no prior history of anemia or gastrointestinal bleeding was admitted to our hospital for elective Coronary Artery Bypass Graft surgery. After surgery, he was started on Aspirin and Plavix. A few days later he developed anemia and heme positive stools. He underwent a colonoscopy which showed numerous large bluish vascular malformations throughout the colon and multiple flat polyps (tubulovillous adenomas). On further inspection, he had bluish nevi on his forearm. These were biopsied and they were consistent with BRBNS.Figure

Profound iron deficiency anemia caused by a small-intestinal cavernous hemangioma

Profound iron deficiency anemia caused by a small-intestinal cavernous hemangioma

Familial Adenomatous Polyposis and Factor VII Deficiency in a Young Man

To report a case of Familial adenomatous polyposis in a young man with concomitant Factor VII deficiency. A 29 y/o African American male presented with complaints of weakness and recent shortness of breath. He attributed the symptoms to several nosebleeds in the past six months. He denied any trauma or other medical history. Patient reported frequent nose bleeds for last six months though denied any vomiting, malena, hematochezia or other sources of bleeding. His father died at age of 42 with colon cancer and uncle also had colon cancer but denied any bleeding disorders in family. He was a smoker, occasional alcohol user but denied illicit drug abuse. On examination, he was tachycardic with blood pressure of 110/50. Rest of the exam was remarkable only for heme positive brown stool on rectal exam. Lab data showed hematocrit 11 with MCV 67, Platelet 473K, TIBC 431, Serum Iron 9 and Ferritin 3 ng/mL. His coagulation profile showed PTT elevated to 20.7 seconds and INR elevated to 1.8. He was admitted and received 3 units of packed red blood cells. With profound iron deficiency anemia and strong familial history of colon cancer, gastrointestinal consultation was sought. Panendoscopy was remarkable for innumerable polyps in colon. FAP was most plausible diagnosis. Biopsies revealed adenomatous polyps without high-grade dysplasia or malignancy. Coagulation parameters did not correct after transfusions. Mixing study showed correction of prothrombin time. Coagulation factor deficiency seemed possible cause. Factor VIII and IX were normal. Factor VII levels was drawn later, after hematology consultation, it was 12 (reference 70–130). A diagnosis of factor VII deficiency was made. He underwent colectomy and received recombinant factor VII perioperatively. Patient was counseled and genetic counseling was advised along with surveillance in other family members. This is an interesting presentation of two rare familial disorders in same patient. Familial adenomatous polyposis is autosomal dominant diseases caused by mutations in the adenomatous polyposis coli on chromosome 5q21. FAP occurs in approximately 1/10000–30000 live births, and accounts for less than 1 percent of the total colon cancer risk in the USA. With estimated prevalence of 1: 500 000, inherited FVII deficiency is also a rare, autosomal recessive coagulation disorders. The FVII gene (F7) is located on chromosome 13. There has been no reported link between these two relatively rare disorders and this might be just a coincidence or not!

A case of profound iron deficiency anemia owing to corrosive esophagitis in a 20-year-old developmentally delayed male

The level of severe compensated iron deficiency anemia incompatible with life is not defined in the pediatric or adolescent literature. A hemoglobin of 1.5 gm/dl in an older adolescent with few physical symptoms is distinctly unusual. A case of profound iron deficiency anemia in a 20-year-old developmentally delayed male is the subject of this brief report. There were only subtle physical findings in spite of this severe anemia. The anemia was the result of corrosive esophagitis associated with a hiatal hernia and reflux. Physicians dealing with developmentally delayed adolescents should be aware of the fact that a severe anemia may develop, and such individuals should be periodically screened for anemia, melena, hematochezia, and hematemesis.

8 - Iron Deficiency in the Tropics

Iron in food is classified as belonging to the haem pool, the nonhaem pool, and extraneous sources. Haem iron is derived from vegetable and animal sources with varying bioavailability. Hookworm infestation of the intestinal tract affects 450 million people in the tropics. Schistosoma mansoni caused blood loss in 7 Egyptian patients of 7.5- 25.9 ml/day which is equivalent to a daily loss of iron of .6-7.3 mg daily urinary loss of iron in 9 Egyptian patients. Trichuris trichiura infestation by whipworm is widespread in children with blood loss of 5 ml/day/worm. The etiology of anemia in children besides iron deficiency includes malaria, bacterial or viral infections, folate deficiency and sickle-cell disease. Severe infections cause profound iron-deficiency anemia in children in central American and Malaysia. Plasmodium falciparum malaria-induced anaemia in tropical Africa lowers the mean haemoglobin concentration in the population by 2 g/dI, causing profound anaemia in some. The increased risk of premature delivery, low birthweight, fetal abnormalities, and fetal death is directly related to the degree of maternal anemia. Perinatal mortality was reduced from 38 to 4% in treated anemic mothers. Mental performance was significantly lower in anemic school children and improved after they received iron. Supplements of iron, soy-protein, calcium, and vitamins given to villagers with widespread malnutrition, iron deficiency, and hookworm infestation in Colombia reduced enteric infections in children. Severe iron-deficiency anemia was treated in adults in northern Nigeria by daily in Ferastral 10 ml, which is equivalent to 500 mg of iron per day. Choloroquine, folic acid, rephenium hydroxynaphthoate, and tetrachlorethylene treat adults with severe iron deficiency from hookworm infestation in rural tropical Africa. Blood transfusion is indicated if the patient is dying of anaemia or is pregnant with a haemoglobin concentration 6 gm/dl. In South East Asia, mg per day prevented iron-deficiency anaemia in pregnancy. Field-trials on nutritional iron deficiency include an acidified milk formula plus ferrous sulphate for infants; biscuits with added bovine hemoglobin for children in Chile; sugar plus sodium ferric EDTA in Guatemala; salt with ferric orthophosphate and sodium acid sulphate in India; and Salt with ferrous sulphate plus sodium hexametaphosphate.

Intracolonic formation of struvite crystals in a patient with congenital megacolon

Intracolonic formation of large crystals and their subsequent rectal passage with hematochezia has not been previously described. We report the case of a patient with Hirschsprung's disease with a long history of anemia and passing crystals in his stool. These crystals were shown, by x-ray diffraction analysis, to be pure struvite (magnesium ammonium phosphate). It is likely that their sharp edges caused rectal bleeding and profound iron-deficiency anemia in this patient.

Lactic Acidosis Associated With Iron Deficiency Anemia

Severe lactic acidosis occurred in a patient with profound iron deficiency anemia. Rapid improvement was observed following the administration of blood, and the patient made a complete recovery.