We present five cases of common variable immunodeficiency (CVID), comprising three women and two men with a mean age of 23.8 ± 9.2 years. All our patients suffered from recurrent bronchopneumonitis, with complications of purulent pleurisy in two cases, requiring decortication in one case, and resulting in bronchiectasis in three cases. Digestive tract infections were observed in four patients, while two patients presented with ENT infections. One case was complicated by bacterial meningitis. All patients presented with global hypogammaglobulinemia, with CVID and granulomatous manifestation in one case. Treatment consisted of monthly immunoglobulin infusions.

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Marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) of the breast with amyloid deposits is a very rare cause of breast malignancy. Patients who carry a diagnosis of Sjogren\'s syndrome (SS) have a 5-10% lifetime risk of developing non-Hodgkin lymphoma with MALT lymphoma as the most common histologic subtype. Our case highlights the importance of routine screening mammography in the early detection of such unusual malignancies, and further interventions needed to diagnose and appropriately manage breast MALT lymphoma.

Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory autoimmune disease characterized by tissue infiltration by dense lymphoplasmacytic infiltrate composed of T cells, activated B-cells, and plasma cells expressing IgG4 and has varied presentations with similar histopathology. It can involve visceral organs, glands, aorta, lymph nodes, and retroperitoneal tissue. In our case, a 68-year-old male with a past history of Hodgkin\'s lymphoma and in remission presented for investigation of polyclonal gammopathy. Serum electrophoresis showed increased free kappa light chains, free lambda light chains, and kappa lambda ratio; immunoglobulin G (IgG) levels were also increased. A positron emission tomography (PET) scan and magnetic resonance imaging (MRI) thoracic spine suggested a hypermetabolic prevertebral soft tissue density. Biopsy of the mass suggested IgG4-related disease (IgG4-RD). He also had a compression fracture of the T7 vertebra. He was started on intravenous methylprednisolone and rituximab, following which he had a significant decrease in the size of the mass along with a decline in the levels of IgG, kappa, and lambda chains.

Multiple myeloma (MM) is an uncommon hematologic malignancy in the general population and extremely rare in pregnant and peripartum women. Here we report a rare case of multiple myeloma in a 43-year-old African American woman at 24 weeks gestation who presented for severe flank pain and difficulty breathing. An elevated D-dimer was present on initial presentation and diagnoses of deep vein thrombosis and pulmonary embolism were ruled out via bilateral Doppler ultrasounds and CT scan. Lytic bone lesions, anemia, and elevated total protein were also noted and an iliac crest biopsy demonstrating monoclonal plasma cells confirmed the diagnosis of MM. She was started on steroids at 27 weeks gestation and delivered via c-section at 30 weeks gestation. Postpartum, a treatment regimen to allow for breastfeeding was discussed but was unable to be accommodated, and she was started on a multi-drug chemotherapy regimen. There have only been about 30 cases of multiple myeloma in pregnant women reported in the literature. The rarity and lack of information on the effects of multiple myeloma in pregnancy was the primary indication for publication of this case.

BACKGROUND: Accurate quantification of immunoglobulin A (IgA) monoclonal immunoglobulins by serum protein electrophoresis (SPEP) can be difficult and can impact the assessment of response among patients with multiple myeloma (MM). Therefore, there is a need to identify new assays that better reflect disease burden and response to treatment, and correlate with patient outcome. IgA Hevylite (HLC) measures IgA kappa and IgA lambda separately and provides precise quantitative measurements of the monoclonal IgA expression and polyclonal-isotype matched suppression. In the current study, the authors assessed the usefulness of these assays in the diagnosis of IgA MM and sought to comment on the prognostic value of the assays.
METHODS: A study of 157 patients with IgA MM for whom diagnostic samples were available was performed. HLC measurements were performed on a nephelometer and the results were compared with those of electrophoresis.
RESULTS: All presentation sera (100 IgA kappa specimens and 57 IgA lambda specimens) were found to have abnormal IgA HLC ratios (IgA kappa median ratio: 336.2 [range, 8.2-7353] and IgA lambda ratio: 0.011 [range, 0.0003-0.45]). In comparison, SPEP bands were quantifiable in only 105 of 157 samples (67%) (median, 28.5 g/L [range, 2.2 g/L-98 g/L]). Of the total of 157 patients, 12 patients (8%) presented with oligosecretory myeloma (<10 g/L; including 4 patients with nonquantifiable SPEP bands). HLC uniquely allows for the measurement of isotype paired suppression, which was found to be associated with shortened overall survival in the current study.
CONCLUSIONS: In the current study, IgA HLC ratios were found to be abnormal in all patients and the assay was able to produce quantifiable results in more MM sera than either SPEP or total IgA, potentially representing a solution to the issue of comigration and oligosecretory MM. These preliminary data require confirmation in larger prospective trials to validate the usefulness of IgA HLC.