RESUMO
Cancer is a major cause of morbidity and mortality worldwide, with squamous cell carcinoma (SCC) being the most common type. Even though SCC is the major type of cancer found in the head and neck region, the salivary glands contribute to about 1/20 cases, of which 1/10 are said to be carcinoma ex pleomorphic adenoma (CXPA) type, and the parotid gland is found to be the most common origin of such cases. Although it usually arises later in life, it can grow rapidly, with local symptoms being late findings, if any. Even though fine needle aspiration cytology has low sensitivity for diagnosing such cancer, multiple/repeated biopsies can increase the yield and the accuracy of the test. Surgical resection is the main choice for treatment with postoperative radiation for select cases. Our case presented with CXPA with distant metastasis to multiple sites.
RESUMO
Systemic mastocytosis is a rare hematologic disorder characterized by the clonal proliferation of mast cells in extra-cutaneous organs. This disease can be further subdivided into five different phenotypes: indolent systemic mastocytosis (ISM), smoldering systemic mastocytosis (SSM), aggressive systemic mastocytosis (ASM), systemic mastocytosis with an associated hematological neoplasm (SM-AHN) and mast cell leukemia (MCL). The tyrosine kinase inhibitor (and also potent KIT D816V inhibitor) avapritinib, initially approved for the treatment of gastrointestinal stromal tumors (GISTs) bearing a PDGFRA exon 18 mutation, also showed great promise in patients with systemic mastocytosis, a disease known to be driven by a mutation in KIT (D816V). We present an overview of this rare disorder, including a review of the current understanding of the genetic mechanisms which lead to the disease state, the action of the tyrosine kinase inhibitors, as well as the latest clinical trial data which led to the current recommendations for the use of avapritinib.
RESUMO
Thrombotic thrombocytopenic purpura (TTP) is a rare but a potentially fatal condition. Although the majority of TTP cases are of unknown etiology, certain viral infections, malignancies, and medications have been linked to the acquired form of the illness. Regardless of the underlying etiology, TTP remains a great challenge diagnostically and therapeutically. TTP remains a very uncommon complication of HIV. We reviewed the current literature to better understand the relationship between HIV and TTP and address some of the major obstacles that may impede or delay the correct diagnosis. Here, we present a case of a 28-year-old male with complaints of light-headedness, fatigue, and gingival bleeding. He was found to have severe anemia and thrombocytopenia. He tested positive for the HIV and was then diagnosed with TTP. Despite needing endotracheal intubation for airway protection, he clinically improved with packed red blood cells, plasmapheresis, and highly active antiretroviral therapy.