Division VI

Abstracts Submitted: Division VI - Case History


CD4 LYMPHOPENIA IN A YOUNG MALE: APPROACH TO THE DIFFERENTIAL DIAGNOSIS .

Halawa A, Myers J, Reynolds S and Krishnaswamy G. Department of Medicine, Divisions of Allergy and Immunology and Infectious Disease, Quillen College of Medicine.

Case Presentation: Patient is 37 year old white male was referred to the Allergy clinic because of CD4 Lymphopenia. Pt had long history of chronic sinusitis (currently on several inhalers by his PCP). A chest roentgenogram demonstrated bilateral infiltrates and hence he was admitted to the hospital because of intermittent cough worsening at night; SOB while running or walking up ramp with bilateral lung infiltrates on CXR. On admission, he was being treated with albuterol inhaler, famotidine, fluticasone nasal spray and acetaminophen. Serum chemistry, liver function, erythrocyte sedimentation rate and complete blood count were all within normal limits. The patient underwent an immune evaluation that revealed the following: CD4+ T cells 109 cells/cu.mm, CD8+ve T cells 77 cells/cu.mm and CD4/CD8 ratio of: 1.38. The findings were consistent with severe CD4 lymphopenia. Materials and Methods: The patients chart was reviewed and appropriate data collected. Results: The differential diagnosis of CD4 lymphopenia include: HIV infection, leukemias and malignancy, iatrogenic cause (glucocorticoids, cytotoxic drugs), Rheumatological disease (Sjogren's syndrome), granulomatous disease (TB, Sarcoidosis), malnutrition (protein-calorie eg., Kwashiorkar) and finally idiopathic CD4 lymphopenia (ICDL). ICDL is a diagnosis of exclusion and the etiology of this condition is uncertain. In our patient, evaluation excluded the presence of HIV, hematological malignancy, rheumatological disease, iatrogenic causes and malnutrition. The computerized tomogram of the chest demonstrated perihilar masses associated with granulomatous calcifications with right middle lobe partial Atelectasis. Pulmonary function demonstrated a restrictive profile. An open lung biopsy demonstrated the presence of noncaseating granulomas. Ocular examination demonstrated no uveitis and fundoscopy was normal. The diagnosis was compatible with stage III sarcoidosis. The patient was treated with prednisone and had some improvement of symptoms. Due to the presence of interstitial fibrosis, complete resolution of disease is unlikely in this patient and he may be a candidate for lung transplantation. His CD4 counts have remained low. Conclusion: a occurred in this patient, compartmentalization of CD4+ T cells in the sarcoid granuloma can lead to peripheral CD4 lymphopenia. The granuloma is initiated by macrophage and T cell activation and interferon gamma secretion. The core of the sarcoid granuloma consists of epithelioid cells and easily discernable multinucleated giant cells. This core is infiltrated and surrounded by a layer of predominantly T-helper (CD4) cells that, in turn, is bounded by a loose aggregation of a cellular core. The CD4 lymphopenia may be rarely complicated by opportunistic infection (such as Cryptpcoccus).


EXCESS THYROID HORMONE AS A RISK FOR CORONARY EVENTS-A CASE REPORT AND LITERATURE REVIEW-

KASASBEH E., M.D., ISKANDAR S., M.D., GARCIA I., M.D.,  FACC, DIVISION OF CARDIOLOGY, DEPARTMENT OF INTERNAL MEDICINE, ETSU, JOHNSON CITY, TN 37614

Myocardial infarction and unstable angina have been reported as complications in patients with hyperthyroidism, even in the setting of normal coronary arteries. The exact involved mechanisms are not clearly defined although some hypotheses have been suggested. We report the case of a 78 year old female patient who presented with seizures secondary to hyperthyroidism that was uncontrolled on maximum anti-thyroid medications. Electrocardiography showed ST-segment depression. Serial cardiac enzymes showed elevation of serum troponin-I. Cardiac catheterization showed normal coronary arteries. In this article, we review all the cases previously reported in the English literature, studying the prevalence, pathogenesis, morbidity and the suggested therapy.


OBSTRUCTIVE SLEEP APNEA AND ITS CARDIOVASCULAR COMPLICATIONS - A PATIENT WITH ASTHMA, OBESITY, AND HYERTENSION

Kasasbeh, E. , MD, Krishnaswamy, G., MD, Department of Internal Medicine, East Tennessee State University, Johnson City, TN 37614.

Obstructive sleep apnea (OSA) is a common medical condition that occurs in a substantial percentage of the population. It has evolved as a disease affecting multiple organs. Many patients have many of these complications. We present the cases of a 58 year old patient with multiple medical problems including hypertension who presents with daytime somnolence, fatigue, and snoring. He developed worsening of his condition and finally sleep study showed obstructive sleep apnea. Sleep study done on CPAP showed that resolution of the apneic episodes. Our objective of was to review the cardiovascular complications that occur secondary to sleep apnea, and to assess the degree to which they affect the development of chronic cardiovascular pathology and disease. The pathophysiology of OSA is characterized by repetitive occlusions of the posterior pharynx during sleep that obstruct the airway, followed by oxyhemoglobin desaturation, persistent inspiratory efforts against the occluded airway, and termination by arousal from sleep, with these apnea spells occurring numerous times during sleep. OSA is associated with daytime sleepiness and fatigue, likely due to fragmented sleep from recurrent arousals. Substantial evidence shows that patients with OSA have an increased incidence of hypertension compared with individuals without OSA and that OSA is a risk factor for the development of hypertension. Recent studies show that OSA may be implicated in stroke and transient ischemic attacks. OSA appears to be associated with coronary heart disease, heart failure, and cardiac arrhythmias. Pulmonary hypertension may be associated with OSA, especially in patients with preexisting pulmonary disease. Although the exact cause that links OSA with cardiovascular disease is unknown, there is evidence that OSA is associated with a group of proinflammatory and prothrombotic factors that have been identified to be important in the development of atherosclerosis. OSA is associated with increased daytime and nocturnal sympathetic activit.....


A Patient with Persistent Pruritis, Esophageal Reflux and Thickened Skin.

Goutham Dronavalli, M.D., Hadi El Bazouni, M.D., Guha Krishnaswamy, M.D. Department of Internal Medicine; Stuart Leicht, M.D., Divsion of Dermatology, George A Youngberg, M.D., and Changlee S. Pang, M.D., Department of Pathology, Quillen College of Medicine, Johnson City, TN, 37604.

A 63 year old Caucasian male presented with persistent pruritis, marked over his forearms. An extensive earlier evaluation at a different practice had provided no unifying diagnosis. The patient also gave a history of esophageal reflux, dyspnea and symptoms compatible with Raynaud’s phenomenon. Examination revealed thickening of the skin of the forearm, face and hands as well as digital ulcers. The findings were compatible with primary systemic sclerosis (PSS). Computerized tomography of the chest demonstrated extensive mediastinal adenopathy. Because of the anecdotal reports of underlying lymphoma or sarcoid complicating PSS, mediastinoscopic biopsy of a lymph node was carried out which demonstrated benign adenopathy. Skin biopsy revealed extensive collagen deposition and a paucity of CD34+ spindle cells in the dermis, all compatible with PSS. PSS can be a localized (linear scleroderma, morphea, eosinophilic fasciitis) or systemic (diffuse, limited). The differential diagnosis of PSS includes bleomycin therapy, vinyl chloride intoxication, diabetes mellitus, amyloidosis and vibration-mediated disease. Fibrosis is a prototypic finding in PSS. The mechanism of fibrosis in the disease is unclear, but mast cell infiltration and degranulation have been demonstrated. Mast cell-derived factors such as tryptase and histamine may play facilitatory roles. T cell, macrophage and mast cell-derived cytokines such as transforming growth factor beta, interleukin-1 (IL-1), IL-4, IL-6, IL-10 and IL-13 may also play a role in the fibrotic aspect of the disease. Complications of PSS include renovascular disease, hypertensive crises, pulmonary hypertension, interstitial lung disease and aspiration. No effective treatment is available for PSS though various empiric therapies have been proposed. This patient was treated with a proton pump inhibitor and an angiotensin receptor antagonist with some improvement of his symptoms. PSS can present as pruritis in some patients. The differential diagnosis of pruritis must also include metabolic conditions, hepatobiliary disease, malignancy, primary dermatological disease as well as autoimmunity.  A careful evaluation of the patient complaining of pruritis can often lead to an underlying diagnosis.


AN UNUSUAL PRESENTATION OF SEVERE MITRAL STENOSIS WITH AN EXTENSIVE ATRIAL THROMBUS

Kais Albalbissi, MD, Said Iskandar, MD, Jonathan Burress, MD, Israel Garcia, MD, FACC, Division of Cardiology, Department of Internal Medicine, James H. Quillen College of Medicine, East Tennessee State University. Johnson City, Tennessee

Mitral stenosis is a well-described valvular heart disease. It commonly presents with exertional dyspnea. It may also present with a variety of other symptoms including hemoptysis, chest pain, atrial fibrillation and systemic thromboembolism. Here we report a 68 year-old patient with an unusual presentation of mitral stenosis. He presented with recurrent episodes of hemorrhagic pleural effusion. Afterward, an extensive atrial thrombosis complicated his course of illness. We will discuss how the clinical presentation of mitral stenosis is mainly dictated by the underlying pathophysiology of the disease. Moreover, the need for anticoagulation in the setting of mitral stenosis is frequently linked to the presence of atrial fibrillation. We will discuss the independent risk factors for thromboembolism in the setting of mitral stenosis. Then, a review of the current recommendation for anticoagulation is conferred.


AN UNUSUAL CASE OF PYOPNEUMOTHORAX IN TENNESSEE

Souad S.Youssef, MD, Vijay Ramu, MD and Felix A.

Sarubbi, Department of Medicine, James H. Quillen College of Medicine, East Tennessee State University, Johnson City, TN, 37614

Rupture of a coccidioidal pulmonary cavity with subsequent pyopneumothorax is a rare clinical event even in areas endemic for coccidioidomycosis.Our encounter with a patient diagnosed with this condition in Northeast Tennessee serves notice to clinician that coccidioidomysosis is indeed a traveling fungus disease and practitioners must be alert to common and uncommon manifestations of infection associated with this fungus. Literature review pertaining to coccidioidal pyopneumonthorax revealed that patients usually present with recent onset chest pain, serologic testing and pleural fluid culture are highly useful and managements includes surgical intervention with or without antifungal therapy.


Dapsone Hypersensitivity Syndrome:A Case Report and Review of the Literature

Semaan G. Kosseifi, MD, Bhuvana Guha, MD, Dima N.Nassour, MD and Guha Krishnaswamy MD., Department of Internal Medicine, Division of Allergy and Immunology

A patient presented with an eruption, fever, pulmonary infiltrates and anemia following the institution of dapsone therapy. Her syndrome was compatible with hypersensitivity to this drug. 4,4’-Diaminodiphenylsulphone (Dapsone) a historical drug throughout generation was described first by Lowe & Smith in 1949 and Alldays & Barnes in1951 (1;2). Dapsone hypersensitivity syndrome (DHS) typically presents with a triad consistent of fever, skin eruption, and internal organ involvement. This syndrome is treated with discontinuation of the drug and intravenous administration of steroids.


ANAPHYLACTIC REACTION TO INTRAVENOUS IMMUNOLGOBULIN (IVIG) IN A PATIENT WITH COMMON VARIABLE IMMUNODEFICIENCY (CVID).

Al Hasan M., M.D., Youness H., M.D., Sakhare R., B.S., and Krishnaswamy G., M.D. Division of Allergy and Clinical Immunology, Department of Medicine, East Tennessee State University.

An elderly Caucasian male with a prior history of repeated infections was evaluated for immune deficiency. Immunological testing demonstrated very low levels of IgG (<100 mg/dL), IgA (<68) and IgM (26). The patient had very poor responses to pneumococcal vaccination. These findings were diagnostic of CVID. CVID is an acquired disorder of the immune system that leads to decreased immunoglobulin levels with resultant complications. These may include recurrent infectious illnesses (bacterial pathogens, mycoplasma and/or pneumocystis carinii), autoimmune syndromes (Sjogrens-like or rheumatoid-arthritis-like syndrome), sarcoid-like disease, sprue and a higher than normal prevalence of malignancy. The therapy for this disorder is IVIG, usually infused in the dose of 400 mg/Kg body weight, sufficient to elevate serum immunoglobulin levels to above 550 mg/dL. Occasional patients with CVID and selective IgA deficiency may demonstrate immediate hypersensitivity reactions to infused IVIG. This is usually mediated by anti-IgA antibodies and can manifest as nausea, diaphoresis, skin eruption, diarrhea and hypotension. The patient in question was initiated on Carimune, an IVIG product that is reasonably enriched in IgA. Within 2 minutes of the infusion, the patient grew diaphoretic and tachycardic. He developed nausea, vomited and had fecal incontinence. The infusion was stopped and the patient treated. Subsequent evaluation demonstrated very high levels of anti-IgA antibodies (of the IgG kind). Because of the potential for life threatening infections without replacement IVIG, the patient was started on subcutaneous infusion of Gammagard, a product known to have miniscule quantities of IgA. This will be given in small doses of 5G twice/week over 4 weeks, initially as an inpatient and subsequently as an outpatient. Patients with CVID or IgA deficiency may develop anti-IgA antibodies (20% and 40% respectively) and a subset of these patients may react to infused blood products including IVIG. Special precautions are often needed in these individuals who pose serious therapeutic and management challenges. The use of IVIG preparations extremely low in IgA content, pretreatment and the use of subcutaneous infusions may all help in management of these patients. The only disadvantage of subcutaneous infusions is the small volume that can be delivered and the frequency of the administrations, which may pose difficulties for working individuals. In selected patients, home subcutaneous infusions may be possible and may lessen the burdens associated with hospital or office infusions.