Rare Complication of a Rare Malignancy: Case Report of Cardiac Amyloidosis Secondary to Waldenstrom Macroglobulinemia.

Cardiac amyloidosis is a rare disorder caused by the myocardial deposition of abnormal fibrils. A 52-year-old man was referred to our center with clinical features of heart failure, after cardiac magnetic resonance imaging showed restrictive cardiomyopathy. Abdominal fat pad biopsy showed features of amyloidosis, and after hematological workup, he was diagnosed with Waldenstrom macroglobulinemia (WM). He was initiated on a rituximab-based chemotherapy regimen, and his cardiac function was assessed serially. Because of non-response, he was switched to a bortezomib-based regimen. Unfortunately, three days into this regimen, the patient died. WM is a rare plasma cell dyscrasia with a nonspecific presentation. It uncommonly presents with sequelae of amyloidosis-the IgM subtype of amyloid-light chain (AL) amyloidosis. Diagnostic delays are common, contributing to an already poor prognosis. Amyloidosis in WM requires urgent treatment - clonal chemotherapy, and supportive cardiac care in heart involvement. Bortezomib-based regimens are commonly recommended, with diuretics as the mainstay for cardiac treatment. However, in most advanced cases, the prognosis is poor; thus, a high degree of suspicion is necessary for early diagnosis. This case illustrates the possible presentation of cardiac amyloidosis as a rare malignancy.

Evaluation of the use of gel card system for assessment of direct coombs test: weighing the pros and cons.

A positive direct Coombs test (DCT) is the hallmark of diagnosis of immune hemolytic anemias. The reagent used for the test is the Antihuman globulin (AHG), which may be either 'Polyspecific' or 'Monospecific'. The advent of the Gel card systems has made the procedure and interpretation of DCT simpler. Aim of this study is to evaluate three of the various techniques used for the performance and interpretation of DCT. A total of 96 EDTA samples were included in the study. DCT was performed by (i) polyspecific AHG manual tube method (ii) polyspecific AHG Gel card method and (iii) monospecific AHG (Anti IgG and Anti Complement) manual tube method. In our study we considered positivity by monospecific AHG as the standard for diagnosis. Of the total 96 samples evaluated, 44 cases positive by Gel card method, were also positive for either one or both the monospecific AHG reagents. 17 cases positive by Gel card were negative by all manual methods. These false positive cases were attributed to reasons such as increased ESR, macrocytosis and marked leucocytosis. Nine cases were negative by Gel card but were positive with the Monospecific AHG. The sensitivity of DCT done by the Gel card technique was 83.01% and the specificity was 60.46%. Use of Gel card technique to perform and interpret DCT is easier than manual tube methods, but positivity by Gel card needs to be correlated with clinical presentation of the patient and other laboratory findings. Monospecific antisera can be used to confirm cases that are positive by the Gel card systems.