Complement-Mediated Thrombotic Microangiopathy: A Murky Presentation of a Rare Disease Entity

Complement-mediated thrombotic microangiopathy (TMA), also known as atypical hemolytic uremic syndrome (aHUS) is a rare, hereditary, progressive, life-threatening disorder caused by a disruption in regulation of the alternative pathway of the complement system. Eculizumab, a terminal complement inhibitor, has emerged as a first-line therapy, however data are limited to small case series (Brocklebank et al., 2017). Here, we present a diagnostically challenging case of complement-mediated TMA, who received eculizumab therapy with excellent hematologic response.A 68-year-old female with history of possible Sjogren's syndrome, migraine disorder, chronic fatigue syndrome, inflammatory colitis, hypertension, and poor medical follow up presented with 6-day history of severe fatigue, hematochezia, decreased urine output, dyspnea with exertion and anginal chest pain. 2 weeks prior, patient endorsed "flu-like" illness and had diffuse myalgias without fevers. Further history revealed ibuprofen usage of 800-1200 mg/day for several years. Shortly after admission, patient became severely agitated and confused with an attempt to elope from hospital.During diagnostic workup, labs were significant for hemoglobin 5.6 g/dL, platelets 57,000/uL, serum creatinine 6.6 mg/dL, BUN 101 mg/dL. Peripheral smear showed schistocytes and tear drop cells, low platelets without clumping, and hypochromic normocytic red cells. LDH of 2152 U/L, haptoglobin
Source: Blood - Category: Hematology Authors: Tags: 311. Disorders of Platelet Number or Function Source Type: research

Related Links:

Authors: Ono Y, Yoshikura N, Takekoshi A, Ohe N, Hayashi H, Yamada M, Hayashi Y, Kimura A, Shimohata T Abstract A 57-year-old woman with amyotrophic lateral sclerosis (ALS) receiving mechanical ventilation developed intractable right temporal headache. She was diagnosed with brain abscess secondary to chronic suppurative otitis media. In this case, the otitis media was caused by nasopharyngeal reflux associated with eustachian tube muscle weakness and a supine position. In addition, ALS patients under mechanical ventilation have a limited ability to convey their pain. Their complaints are often overlooked because m...
Source: Internal Medicine - Category: Internal Medicine Tags: Intern Med Source Type: research
Authors: Ichijo M, Tsuchiya K, Kasai T, Inoshita N, Yoshimoto H, Yamada S, Kitamura K Abstract A 30-year-old woman with multiple ovarian cysts presented with high serum estradiol levels. She had a pituitary adenoma, but the follicle-stimulating hormone (FSH) concentration was within the normal range. The patient complained of neck pain and palpitations during the disease course, and laboratory results revealed thyrotoxicosis and a systemic inflammatory response with negative findings for anti-thyroid stimulating hormone (TSH) receptor antibody and positive findings for anti-thyroglobulin and anti-thyroid peroxidase...
Source: Internal Medicine - Category: Internal Medicine Tags: Intern Med Source Type: research
Authors: Usami J, Takezawa Y PMID: 31611529 [PubMed - as supplied by publisher]
Source: Internal Medicine - Category: Internal Medicine Tags: Intern Med Source Type: research
ConclusionsExternal beam APBI results in excellent long-term disease control. Good to excellent cosmetic outcomes are achieved in most patients, although increasing dose per fraction and greater percentage of irradiated breast were predictive of adverse posttreatment cosmetic outcomes. Select patients in “cautionary” and “unsuitable” consensus groupings do not appear to have inferior outcomes.
Source: Practical Radiation Oncology - Category: Cancer & Oncology Source Type: research
ConclusionsThe exposure –response analysis suggested that intermittent 7-day veliparib 120 mg BID dosing in a 21-day cycle provided additional efficacy without meaningfully impacting the safety and tolerability when co-administered with carboplatin and paclitaxel in patients with BRCA-deficient breast cancer. A higher d ose of veliparib is unlikely to provide greater benefit in this combination in patients with BRCA-deficient recurrent or metastatic breast cancer.
Source: Cancer Chemotherapy and Pharmacology - Category: Cancer & Oncology Source Type: research
No abstract available
Source: Topics in Pain Management - Category: Anesthesiology Tags: CONTINUING EDUCATION ACTIVITY Source Type: research
No abstract available
Source: Topics in Pain Management - Category: Anesthesiology Tags: ARTICLE Source Type: research
No abstract available
Source: Topics in Pain Management - Category: Anesthesiology Tags: ARTICLE Source Type: research
No abstract available
Source: Topics in Pain Management - Category: Anesthesiology Tags: CE Quiz Source Type: research
No abstract available
Source: Topics in Pain Management - Category: Anesthesiology Tags: NEWS IN BRIEF Source Type: research
More News: Anemia | Autoimmune Disease | Brain | Cancer & Oncology | Chronic Fatigue Syndrome | Chronic Pain | Corticosteroid Therapy | Dialysis | Gastroenteritis | Genetics | Headache | Hematology | Hemodialysis | Hemolytic Uremic Syndrome (HUS) | Hospitals | Hypertension | Ibuprofen | Microangiopathic Hemolytic Anemia | Microcytic Hypochromic Anemia | Migraine | Neurology | Normocytic Normochromic Anemia | Pain | Papanicolaou (Pap) Smear | Pre-eclampsia | Pregnancy | Rare Diseases | Renal Failure | Sjogren's Syndrome | Thrombocytopenia | Transplants