Indolent Systemic Mastocytosis Manifesting as Protracted Anaphylactic Shock.

Systemic mastocytosis is a rare disease due to abnormal proliferation of mast cells (MCs). A case of indolent systemic mastocytosis is presented here. After anesthetic induction for elective thyroid swelling with propofol and atracurium followed by endotracheal intubation, a 57-year-old female patient developed acute hypotension, sinus tachycardia, red rashes, increased airway pressure along with difficult ventilation, and desaturation. She developed multiorgan failure subsequently. MC tryptase level was persistently high. Bone marrow study revealed mastocytosis. She required antihistaminic, steroid, and organ support. With treatment, organ functions recovered gradually. Atracurium precipitated anaphylactic shock causing severe morbidity in this patient.

MYD88 and CXCR4 Mutation Profiling in Lymphoplasmacytic Lymphoma/Waldenstrom's Macroglobulinaemia.

Recurrent mutations affecting MYD88 and CXCR4 gene nowadays form the basis for the diagnosis, risk stratification and use of inhibitors targeting these signalling pathways in LPL/WM which are rare B cell neoplasms. MYD88 L265P mutation analysis was performed on 33 cases of LPL/WM by AS-PCR (positivity-84.8%, n = 28/33) and by Sanger sequencing (positivity-39.3%, n = 13/33). We had only two cases with CXCR4 non-sense (NS) mutation (p.S338*) using Sanger sequencing. MYD88 (L265P) mutation detection by AS-PCR can form reliable biomarker for the diagnosis of LPL/WM in molecular labs. Although the cohort is small, still the CXCR4 mutation frequency in our study is low as compared to the published literature.

Stensen Duct Relapse of Diffuse Large B-Cell Lymphoma: An Unusual Pattern Detected With 18F-FDG PET/CT.

F-FDG PET/CT is now an established modality for staging and restaging high-grade lymphomas, including diffuse large B-cell lymphoma. We here present the case of a 51-year-old woman successfully treated for diffuse large B-cell lymphoma. Follow-up F-FDG PET/CT demonstrated an unusual site of relapse involving left Stensen (parotid) duct, which was confirmed with cytology. By identifying such unusual sites of lymphomatous involvement, F-FDG PET/CT can have significant bearing on patient management.

Clinical and serological characterization of cold agglutinin syndrome in a Tertiary Care Hospital in Eastern India.

BACKGROUND AND AIM: Cold agglutinin syndrome (CAS) primary or secondary represents approximately 16-32% of autoimmune hemolytic anemia cases. Most patients present with mild, chronic hemolytic anemia with exacerbation of the condition in the cold environment. Red cell transfusions are only indicated when there is a life-threatening anemia causing crisis. We studied the clinical and serological characterization of CAS with the aim that the information gained from this study would help in proper diagnosis and management of these patients. MATERIALS AND METHODS: The prospective study included nine patients who were admitted with severe anemia. Detailed work-up were conducted to establish the diagnosis, severity of in vivo hemolysis and transfusion management. RESULTS: All patients presented with pallor, weakness, fatigue and painful fingers and toes with exacerbation of symptoms in winter months. Secondary CAS was observed in three patients suffering from malignant lymphoma. Red cells of all patients were coated with complements (C3) more specifically C3d. In one patient suffering from malignant lymphoma, the cold autoagglutinin titer was as high as 4096. Autoantibody in seven patients was specific to "I" antigen and one to "i" antigen. CONCLUSIONS: We conclude that detailed clinical and serological characterization is needed to diagnose and manage CAS. Whereas avoidance of cold exposure is the primary therapy, but no critical patient should be denied blood transfusion due to serological complications. All transfusion services should follow the correct protocol to maximize blood safety in CAS.