scholarly journals Fatal liver mass rupture in a common-variable-immunodeficiency patient with probable nodular regenerating hyperplasia

2022 ◽  
Vol 18 (1) ◽  
Author(s):  
Mongkhon Sompornrattanaphan ◽  
Ranista Tongdee ◽  
Chamard Wongsa ◽  
Anupop Jitmuang ◽  
Torpong Thongngarm
Keyword(s):  
Portal Hypertension ◽  
Common Variable Immunodeficiency ◽  
Multiple Organ ◽  
Late Complications ◽  
Liver Mass ◽  
Cvid Patient ◽  
Hepatic Rupture ◽  
Fatal Liver ◽  
First Case ◽  
Common Variable

Abstract Background Nodular regenerating hyperplasia (NRH) is the most common liver involvement in common variable immunodeficiency (CVID). Most patients are asymptomatic with gradually increasing alkaline phosphatase (ALP) and mildly elevated transaminase enzymes over the years. We report the first case of fatal liver mass rupture in a CVID patient with probable NRH. Case presentation A 24-year-old man was diagnosed with CVID at the age of 1.25 years. Genetic testing revealed a transmembrane activator and calcium-modulator and cyclophilin-ligand interactor (TACI) mutation. He had been receiving intravenous immunoglobulin (IVIg) replacement therapy ever since then. The trough level of serum IgG ranged between 750–1200 mg/dL. However, he still had occasional episodes of lower respiratory tract infection until bronchiectasis developed. At 22 years old, computed tomography (CT) chest and abdomen as an investigation for lung infection revealed incidental findings of numerous nodular arterial-enhancing lesions in the liver and mild splenomegaly suggestive of NRH with portal hypertension. Seven months later, he developed sudden hypotension and tense bloody ascites. Emergency CT angiography of the abdomen showed NRH with intrahepatic hemorrhage and hemoperitoneum. Despite successful gel foam embolization, the patient died from prolonged shock and multiple organ failure. Conclusions Although CVID patients with NRH are generally asymptomatic, late complications including portal hypertension, hepatic failure, and hepatic rupture could occur. Therefore, an evaluation of liver function should be included in the regular follow-up of CVID patients.

Granuloma-like lesion at subcutaneous immunoglobulin site in a common variable immunodeficiency patient

Immunotherapy ◽  
2019 ◽  
Vol 11 (14) ◽  
pp. 1177-1180 ◽  
Author(s):  
Ankmalika Gupta ◽  
Beverly Wang ◽  
Sudhir Gupta
Keyword(s):  
Intravenous Immunoglobulin ◽  
Common Variable Immunodeficiency ◽  
Immunoglobulin Therapy ◽  
Subcutaneous Immunoglobulin ◽  
Primary Antibody Deficiencies ◽  
First Case ◽  
Granulomatous Lesions ◽  
Cutaneous Granuloma ◽  
Common Variable ◽  
Switching Treatment

Immunoglobulin therapy is the main stay in the treatment of primary antibody deficiencies. Granulomatous lesions are common complication in patients with common variable immunodeficiency (CVID). We present the first case of cutaneous granuloma-like lesion at site of subcutaneous immunoglobulin injections in a patient with CVID. These lesions resolve overtime following switching treatment to intravenous immunoglobulin. Unlike granulomas associated with CVID, granulomatous lesion in this patient did not require any specific therapy, and resolved over a period of 4 weeks following switching subcutaneous immunoglobulin to intravenous immunoglobulin.

scholarly journals Common Variable Immunodeficiency Presenting with Recurrent Ascending Cholangitis Treated with Oral Immunoglobulins

2020 ◽  
Vol 1 (1) ◽  
pp. 11-13
Author(s):  
Marija Rowane ◽  
Jaimin Patel ◽  
Tina Abraham ◽  
Jason Schend ◽  
Robert Hostoffer
Keyword(s):  
Common Variable Immunodeficiency ◽  
Primary Immune Deficiency ◽  
Normal Ranges ◽  
First Case ◽  
Serum Iga ◽  
Ascending Cholangitis ◽  
History Of ◽  
Irritable Bowel ◽  
Duct Stricture ◽  
Common Variable

Abstract Background: Common variable immunodeficiency (CVID) is a heterogeneous group of primary immune deficiency disorders that may be characterized by heightened susceptibility to gastrointestinal (GI) infection. GI conditions manifest in 20 to 50% of CVID patients but rarely include cholangitis. Methods/Results: This is a 61-year-old female, with a history of cholecystectomy and gastric bypass, who presented with recurrent ascending cholangitis for eight years. After a hepaticojejunostomy to correct a bile duct stricture complication from the cholecystectomy, ascending cholangitis was diagnosed by clinical presentation of fever and right upper quadrant pain and imaging revealing pneumobilia. CVID was diagnosed after no response to pneumococcal polysaccharide vaccine, as well as serum IgA and IgG measured below the normal ranges. The patient was prescribed a successful, weekly regimen of 15 g of oral intravenous immunoglobulin (IVIG) ten percent liquid (Gammaplex® 10%, 5 gm/50 mL). Conclusion: Oral human IVIG is a novel treatment and has been infrequently utilized for management of chronic rotavirus, necrotizing enterocolitis, diarrhea, HIV enteropathy, irritable bowel syndrome, and malnutrition. This is the first case of recurrent ascending cholangitis as the primary manifestation of CVID, as well as successful treatment of this condition with oral IVIG, in the literature.

scholarly journals Retrospective Analysis of a Longitudinal Single Institution 219 Common Variable Immunodeficiency Patient Cohort

2021 ◽  
Author(s):  
Melissa D Gans ◽  
Brian Goodman ◽  
Larry Bernstein ◽  
Rachel Eisenberg ◽  
Arye Rubinstein
Keyword(s):  
Common Variable Immunodeficiency ◽  
Chart Review ◽  
Retrospective Chart Review ◽  
Lymphoid Hyperplasia ◽  
The Novel ◽  
Limited Data ◽  
Single Institution ◽  
Cvid Patient ◽  
Patient Cohort ◽  
Common Variable

Abstract Common variable immunodeficiency is a heterogenous disorder of the immune system associated with immunodeficiency, lymphoproliferation, autoimmunity, and malignancy. Certain laboratory characteristics of common variable immunodeficiency patients can be associated with an increase of related clinical sequelae, though there is limited data on predictive characteristics for clinical sequelae in CVID patients. The purpose of this study was to analyze a unique large CVID patient cohort for predictive laboratory characteristics of clinical sequelae. A retrospective chart review was performed of a longitudinal cohort of CVID patients treated at a single institution, largely by a single provider with higher dose replacement immunoglobulin, that has not been previously described. 219 CVID patients were followed for 1,990 patient-years. 86% of the patients were on immunoglobulin with an average IgG trough of 1260 mg/dL. Low IgG at time of diagnosis, low CD19 absolute cell count, and poor mitogen induced lymphocyte proliferation were associated with increase of CVID clinical sequelae. In particular, this cohort demonstrates the novel finding that low IgG at time of diagnosis prior to immunoglobulin replacement is associated with a higher incidence of lymphoma, bronchiectasis, granulomatous disease, lymphoid hyperplasia, splenomegaly, and hepatic disease.

scholarly journals A rare case of peliosis hepatis in primary immune deficiency

2020 ◽  
Vol 8 ◽  
pp. 2050313X2093199
Author(s):  
Evelyn Angulo ◽  
Sydney Joyner ◽  
Nasma K Majeed ◽  
Sharmilee Nyenhuis
Keyword(s):  
Common Variable Immunodeficiency ◽  
Rare Condition ◽  
Liver Function Tests ◽  
Peliosis Hepatis ◽  
Primary Immune Deficiency ◽  
First Case ◽  
Regenerative Nodules ◽  
Elevated Liver Function Tests ◽  
Underlying Mechanisms ◽  
Common Variable

Peliosis hepatis is a rare condition characterized by blackish-blue blood-filled cavities in hepatic parenchyma caused by dilatation of hepatic sinusoids. Peliosis hepatis has been described in secondary immunodeficiencies and certain medications. We present the first case of peliosis hepatis in a patient with a primary immunodeficiency, common variable immunodeficiency. A 44-year-old African-American male presented with gastrointestinal bleeding and elevated liver function tests. His medical history included common variable immunodeficiency and chronic kidney disease. The patient had jaundice, regenerative nodules on liver pathology, and low immunoglobulin levels. A magnetic resonance imaging of the abdomen with contrast revealed a cirrhotic liver, a 5 × 3 cm lesion, and poorly defined nodules which had decreased enhancement. A computed tomography-guided liver biopsy revealed peliosis hepatis, focal nodular hyperplasia, and fibrosis. No other etiology of his liver disease was found. The etiology of peliosis hepatis in patients with primary immunodeficiencies remains unclear. Additional studies are needed to understand the underlying mechanisms.

scholarly journals Infection after cyanoacrylate injection: a fatal complication in a patient with portal hypertension associated with common variable immunodeficiency

Endoscopy ◽  
2007 ◽  
Vol 39 (S 1) ◽  
pp. E180-E181 ◽  
Author(s):  
O. Lidove ◽  
A. Gervais ◽  
M. Malphettes ◽  
A. Sauvanet ◽  
J.-L. Moretti ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Common Variable Immunodeficiency ◽  
Fatal Complication ◽  
Common Variable

scholarly journals Posner-Schlossman Syndrome in Common Variable Immunodeficiency

2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Madiha Huq ◽  
Neha Sanan ◽  
Phuong Daniels ◽  
Robert Hostoffer
Keyword(s):  
Intraocular Pressure ◽  
Common Variable Immunodeficiency ◽  
Anterior Uveitis ◽  
Rare Condition ◽  
Elevated Pressure ◽  
Normal Limits ◽  
First Case ◽  
Autoimmune Conditions ◽  
The Right ◽  
Common Variable

Introduction. Posner-Schlossman syndrome (PSS) is a rare glaucomatocyclitic crisis with clinical features including recurrent episodes of unilateral elevated intraocular pressure. Autoimmune and infectious causes have been proposed as potential etiologies of PSS. We report the first case of PSS in the setting of common variable immunodeficiency (CVID). Case Report. A sixty-two-year-old Caucasian female with a medical history of CVID and ulcerative colitis presented to the emergency room with complaints of acute right-sided vision changes. She reported image distortion, blurriness, and loss of central vision. Physical exam was significant for mildly injected right conjunctiva, visual acuity of 20/70 in right eye, and 20/25 in left eye. The right intraocular pressure was measured at 34 mmHg and left at 12 mmHg. The gonioscopy and dilated fundus examination were unremarkable. Cup to disc ratio was within normal limits, and no afferent pupillary defects were recorded. The patient was acutely treated with three rounds of dorzolamide/timolol and 0.2% brimonidine which decreased the right eye intraocular pressure to 24 mmHg. On follow-up exam with an ophthalmologist, anterior uveitis including an elevated pressure of 41 mmHg on the right and 18 mmHg on the left eye was noted and a PSS diagnosis was confirmed. Conclusion. PSS remains a rare condition with uncertain etiology and no associated systemic conditions. PSS has been postulated to be linked to autoimmune conditions. CVID is associated with many autoimmune disorders including Sjogren’s, rheumatoid arthritis, and colitis. There have been a few reported CVID-associated ocular diseases including granulomatous uveitis and conjunctivitis, chronic anterior uveitis, and birdshot retinopathy. We describe the first case of PSS in a patient with CVID.

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