Click here to load reader

A Rare Association of Autoimmune Hemolytic Anemia with ... · PDF fileA Rare Association of Autoimmune Hemolytic Anemia with ... anemia, ” American Journal ... tric cancer appeared

  • View

  • Download

Embed Size (px)

Text of A Rare Association of Autoimmune Hemolytic Anemia with ... · PDF fileA Rare Association of...

  • Case ReportA Rare Association of Autoimmune Hemolytic Anemia withGastric Adenocarcinoma

    Kavita Agrawal1 and Flores Alfonso2

    1Department of Internal Medicine, Overlook Medical Center, Summit, NJ 07901, USA2Department of Pathology, Overlook Medical Center, Summit, NJ 07901, USA

    Correspondence should be addressed to Kavita Agrawal; [email protected]

    Received 5 July 2017; Accepted 30 August 2017; Published 9 October 2017

    Academic Editor: Josep M. Ribera

    Copyright 2017 Kavita Agrawal and Flores Alfonso. This is an open access article distributed under the Creative CommonsAttribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work isproperly cited.

    An 80-year-old male presented with dyspnea on exertion for at least two months. He also complained of progressive dysphagia andweight loss of 35 pounds over the last eight months. Initial blood tests showed hemoglobin of 6.1 g/dl, reticulocytes count of 19.7%,total bilirubin of 3.2mg/dl, lactate dehydrogenase of 600U/L, and haptoglobin of less than 8mg/dl, and direct Coombs test waspositive for warm immunoglobulin G. The impression was autoimmune hemolytic anemia (AIHA). The evaluation of dysphagiawith esophagogastroduodenoscopy revealed a single irregular 4 cmmalignant appearing ulcerated mass at the incisura angularis ofthe stomach. The mass was confirmed as adenocarcinoma on biopsy. Diagnostic laparoscopy was positive for malignant cells andhe was diagnosed with stage IV adenocarcinoma of the stomach. Other extensive workup to determine the etiology of AIHA wasnegative (described in detail below). Surgery was deferred primarily due tometastasis of cancer. Initially, hemoglobin was stabilizedby intravenous methylprednisolone, high dose immunoglobulins, and packed red blood cell transfusions. After a few weeks,hemoglobin started trending down again. The patient was weaned off steroids and paradoxically IgG-mediated autohemolysis wascontrolled with the initiation of palliative chemotherapy. Our case highlights a rare occurrence of AIHA in association with gastricadenocarcinoma.

    1. Case Report

    An 80-year-old African American male presented with aninsidious onset of dyspnea on exertion for at least twomonthswith progressive worsening over two to three weeks. It wasalso associated with orthopnea and lower extremity swelling.Prior to this presentation, he used to walk one block orone flight of stairs without getting short of breath. Presently,however, he had difficulty walking even 30 feet on levelground or climbing few steps of a stair.

    He also complained of difficulty swallowing for eightmonths. Initially noticed with solid foods, it had progressedsuch that, now, even liquids had to be swallowed slowly. Henoted that he was unable to swallow pills; this made himfeel like a pill is stuck in the middle of the chest and sohe stopped taking his medications. He also reported a 35-pound weight loss over the last eight months. He deniedodynophagia, nausea, vomiting, constipation, or abdominal

    pain. He denied rash, arthralgias, photosensitivity, dry eyes,dry mouth, joint swelling, or family history of an autoim-mune or rheumatologic disease.

    He had past medical history of hypertension. He denieda prior history of anemia or blood transfusions. He had nopast surgical history. He never had an upper endoscopy orcolonoscopy.He had no known allergies. His onlymedicationwas amlodipine, which he stopped taking eight monthsearlier due to dysphagia. He had a smoking history of 5pack-years but had stopped smoking 30 years ago, he hadoccasional alcohol use of 1-2 glasses of wine during weekends,and he denied illicit drugs use. He had no significant familyhistory. He had not seen his primary care doctor in at least ayear.He lived alone at home andwas independent in activitiesof his daily living.

    Physical examination revealed a thin cachectic male withno apparent distress. His pulse was 76 beats per minute,blood pressure 159/80mmHg, respiratory rate 19 breaths per

    HindawiCase Reports in Oncological MedicineVolume 2017, Article ID 8414602, 5 pages
  • 2 Case Reports in Oncological Medicine

    minute, and oxygen saturation 100% on two-liter nasal can-nula. His body mass index was 19.9 kg/m2. Pale conjunctivaand icteric sclerawere noted.Therewas no lymphadenopathy.Minimal bibasilar crackles were auscultated on lung exam.Heart sounds were normal and rhythm was regular. Nomurmurs were heard. The abdomen was soft, nontender,and nondistended with no hepatosplenomegaly. On bilaterallower extremities, 1+ pitting ankle edema was present. Norash or joint swelling was present.

    Investigations (refer to Table 1) revealed a hemoglobinlevel of 6.1 g/dl which dropped to 5.1 g/dl in the next 12hours with no fluids, white blood cell count of 6160/l,platelet count of 348 103/l, mean corpuscular volumeof 113.8 fl, and a reticulocyte count of 19.1%. Peripheralsmear showed moderate red cell anisopoikilocytosis andpolychromasia. Neutrophils, lymphocytes, and platelets weremorphologically normal.

    Further workup showed total bilirubin of 3.2mg/dl, con-jugated bilirubin of 1.2mg/dl, serum lactate dehydrogenaseof 600U/L, and haptoglobin of less than 8mg/dl, and directCoombs test was positive for warm immunoglobulin G.Serum protein electrophoresis with immunofixation revealedno abnormal monoclonal protein. Rapid human immunode-ficiency virus test was negative. Anti-nuclear antibodies werepositive, but other antibodies as shown in Table 1 were allnegative. As mentioned above, there was no history or examfindings suggestive of rheumatologic diseases. Therefore, thepositive ANA titer was considered incidental. Mycoplasmaantibodies were also negative. Other test results are shownin Table 1. A diagnosis of warm IgG-mediated autoimmunehemolytic anemia (AIHA) was made.

    On day two of hospitalization, further tests were done torule out underlying lymphoproliferative disorders likely con-tributing to AIHA. Computed tomography of the abdomenand pelvis with oral and intravenous contrast showed nofrank evidence of lymphoproliferative disease. Computedtomography of the chest with intravenous contrast revealedan anterior mediastinal soft tissue mass with dystrophic cal-cifications, bilateral pleural effusions, and mediastinal lym-phadenopathy. Considerations for soft tissue mass includedthymic neoplasm and lymphadenopathy.

    Bronchoscopy was performed on day six of hospitaliza-tion. Endobronchial ultrasound was used to perform biopsyof the anterior mediastinal soft tissue mass and subcarinaland mediastinal lymph nodes. Histological review showedcells consistent with lymphnode sampling but nomalignancywas identified. Flow cytometry from the biopsy showed noevidence of malignancy.

    Dysphagia was worked up on day eight of hospitaliza-tion with an esophagogastroduodenoscopy. At 25 cm fromthe incisors, a tight benign appearing esophageal stricturecausing severe obstruction was encountered.This was dilatedusing 8, 10, and 11mm balloon dilators. The obstruction wasthen able to be traversed by scope. At the incisura angularisof the stomach, a single 4 cm mass was encountered. Ithad irregular margins and an ulcerated surface and did nothave any bleeding. The gastroesophageal junction, pylorus,duodenal bulb, and second part of the duodenum appearedto be normal. A biopsy from an ulcerated mass was taken.

    Figure 1: Stomach incisural mass biopsy on lowmicroscopic powershowing gastric mucosa with intestinal metaplasia and involvementby adenocarcinoma with invasion into the submucosa.

    Figure 2: Stomach incisural mass biopsy on high microscopicpower showing disorganized groups of pleomorphic cells withnuclear atypia, large nucleoli, and mitotic activity.

    Pathological review of the incisural mass biopsy revealednuclear atypia, mitotic activity, andmucinous cells in clusterswithout clear epithelial formations invading the submucosa(Figures 1 and 2). A diagnosis of invasive adenocarcinomawas made. The adenocarcinoma of the stomach was furtherstaged in the third week after the initial presentation. A diag-nostic laparoscopy with peritoneal washings was performed.Cytology from the washings was positive for malignant cellsconsistent with metastatic adenocarcinoma. A diagnosis ofstage IV adenocarcinoma of the stomach was made. Giventhe findings from the laparoscopy, the carcinoma was felt tobe nonresectable.

    2. Treatment and Patient Course

    On the day of presentation, after the diagnosis of AIHAwasmade, he was started on intravenousmethylprednisolone1mg/kg body weight daily for five days and was transfusedwith cross-matched compatible packed red blood cells. Ini-tially, his hemoglobin improved from 5.1 g/dl to 8.6 g/dl withthe above regimen, but on day five of hospitalization, hishemoglobin gradually started declining even on steroids. Byday seven, his hemoglobin had dropped to 6.6 g/dl.Therefore,he was started on high dose intravenous immunoglobulin

  • Case Reports in Oncological Medicine 3

    Table 1: Laboratory data.

    On admission 48 hours after admission Reference rangeWhite cell count (per l) 6160 8810 450011000Hemoglobin (g/dl) 6.1 8.6 14.017.0 (men)Hematocrit (%) 18.2 24.4 39.050.0Red cell count (per pl) 1.60 2.46 4.205.70Mean corpuscular volume (fl) 113.8 99.2 80.299.4Platelet count (per l) 363,000 380,000 150,000450,000Differential count (%)Neutrophils 66.7 40.080.0Lymphocytes 27.9 15.040.0Monocytes 4.9 4.012.0Basophils 0.2 0.02.0Eosinophils 0.0 0.100.20Sodium (mmol/l) 137 135 135145Potassium (mmol/l) 3.5 3.8 3.24.9Chloride (mmol/l) 103 99 95110Carbon dioxide (mmol/l) 24 29 2132Glucose (mg/dl) 100 113 70100Blood urea nitrogen (mg/dl) 14 18 718Creatinine (mg/dl) 1.3 1.1 0.61.3Calcium (mg/dl) 7.8 8.0 8.510.2Total protein (g/dl) 6

Search related