Anesthetic Management of a Patient With Mast Cell Activation Syndrome: A Case Study.

Mast cell activation syndrome (MCAS) is a relatively new diagnosis for a constellation of symptoms with sometimes devastating results for patients. A 40-year old woman with MCAS underwent arthroscopic repair of her right shoulder, with successful anesthetic management. This case report discusses the basic immunologic physiology surrounding this syndrome, myriad medications often used by this patient population, and the anesthetic management of this patient. With additional knowledge of this disorder, exposure to its clinical presentation in the perioperative setting, and anesthetic considerations specific to MCAS, the Certified Registered Nurse Anesthetist will be better equipped to effectively manage the complex requirements of this patient population.

[Surgical interventions in patients with systemic mast cell activation disease : Recommendations for perioperative management]. / Chirurgische Eingriffe bei Patienten mit systemischer Mastzellaktivierungserkrankung : Empfehlungen für das perioperative Management.

BACKGROUND: Systemic mast cell activation disease (MCAD, prevalence 5-10%) is a multifactorial, polygenic disease with multisystemic symptoms that is characterized by an unregulated increased release of mast cell mediators and an accumulation of activated mast cells potentially in all organs and tissues. Due to the high prevalence of the disease, physicians involved in surgical, anesthesiological and interventional procedures are often unknowingly faced with MCAD patients experiencing unexpected preoperative, intraoperative and postoperative complications, if no mast cell-specific treatment regimens have been applied. OBJECTIVE: The findings from a literature search, consensus recommendations of the various international expert groups and extensive own experience in the treatment of MCAD patients enable an empirical and evidence-based care of MCAD patients in association with invasive procedures. RESULTS AND CONCLUSION: Due to the high prevalence of MCAD in the population, it can be assumed that patients with MCAD are correspondingly frequently represented in the surgical patient collective. When MCAD-specific peculiarities are preventively considered in the anesthesiological and surgical procedures in patients with proven or suspected mast cell disease, MCAD patients should not be classified as being at risk.

Systemic mastocytosis with associated myeloproliferative neoplasm with t(8;19)(p12;q13.1) and abnormality of FGFR1: report of a unique case.

Systemic mastocytosis is a neoplastic proliferation of mast cells that frequently presents with associated clonal hematological non-mast cell lineage disease. Myeloid and lymphoid neoplasms with abnormalities of the FGFR1 gene are a heterogenous group of rare and aggressive hematopoietic stem cell disorders. About a dozen of chromosome changes involving the FGFR1 gene, presenting as myeloid or lymphoid neoplasms, have been described in the literature. To date, only 2 cases of myeloid and lymphoid neoplasms with abnormalities of the FGFR1 gene have been reported in association with systemic mastocytosis, one with t(8;13) and one with t(8;17) involving the FGFR1 gene. Here we describe another case of myeloproliferative neoplasm with chromosome translocation t(8;19) involving FGFR1 gene associated with systemic mastocytosis.

Mastocytosis of the female breast.

A subset of patients with systemic mastocytosis may manifest with extracutaneous involvement. To the best of our knowledge, mastocytosis of the human breast has not been described. This study reports a case with mastocytosis involving the breasts of a 33-year-old woman associated with mammary hypertrophy (breast mastocytosis). The potential for infiltrating mast cells to mimic lobular carcinoma is emphasized and the relationship to breast hypertrophy in this case is discussed.

Portal vein thrombosis after laparoscopic splenectomy for systemic mastocytosis: a case report and review of the literature.

INTRODUCTION: Laparoscopic splenectomy has become the surgical procedure of choice for various diseases of the spleen. Portal vein thrombosis (PVT) after splenectomy occurs in 0.5% to 22% of patients. Symptoms are nonspecific and include fever, abdominal pain, and epigastric distress. Risk factors for PVT after splenectomy include underlying hematologic disorders, massive splenectomy, and other hypercoagulable states. METHODS: We describe a case of PVT in a woman who underwent laparoscopic splenectomy for symptomatic splenomegaly secondary to systemic mastocytosis. The patient was discharged from the hospital without anticoagulation and experienced nonspecific symptoms beginning 10 days postoperatively. Diagnosis of PVT was made by contrast-enhanced abdominal computed tomography. The patient had no underlying risk factors. Anticoagulation treatment facilitated recanalization of the portal vein and this was verified by Doppler ultrasound at follow-up. CONCLUSIONS: PVT after laparoscopic splenectomy is not uncommon. Signs and symptoms are vague and require a high index of suspicion for timely diagnosis. Anticoagulation is the treatment of choice and allows recanalization of the portal system in the majority of cases.

Total hip replacement in a patient with systemic mastocytosis: a case report.

Systemic mastocytosis is a rare condition that often involves the bone marrow. We report the case of a patient with systemic mastocytosis who underwent total hip replacement. Technical difficulties encountered during the procedure included a narrow medullary canal and abnormally hard bone, later confirmed by laboratory measurements. Follow-up at five years showed a good clinical and radiological outcome.