Multiple Myeloma with Foamy Mott Cells

Intracytoplasmic assorted vacuoles containing immunoglobulin collections are occasionally seen in multiple myeloma. When abundant, they impart a foamy appearance to the tumor cells, which is a potential source for diagnostic pitfalls. Herein, we report the case of a patient who presented with skeletal pain and CT confirmed lytic lesions. A bone marrow biopsy revealed multiple myeloma with unusual foamy Mott cells. The patient was subsequently treated with four cycles of cyclophosphamide, bortezomib, and dexamethasone induction therapy, followed by 3 cycles of lenalidomide with dexamethasone. A biopsy performed following initial biological and immunomodulatory drugs revealed different morphological and clonal characteristics. These features were modified again, five years later, and again, after two years of close monitoring. Hematopathologists should be aware of this morphologic variant of myeloma as well as for the capacity of clonal characteristics, such as light chain monotype, to fluctuate subsequent to treatment.

Evaluation of malnutrition status and clinical indications in children with celiac disease: a cross-sectional study

Background Celiac Disease (CD) is an autoimmune systemic disorder triggered by gluten in genetically susceptible individuals, which can lead to chronic malabsorption. Considering the changes in the manifestations of CD, this study aimed to determine anthropometric indices and clinical indications in children with CD. Methods This cross-sectional study aimed to evaluate the children with CD who had referred to Imam Reza Celiac Clinic between 2016 and 2019. Totally, 361 children were eligible and their anti-tissue transglutaminase (TGA-IgA) level, weight, height, and Body Mass Index (BMI) were extracted from their records. The anthropometric indices were presented based on the criteria of the Center for Disease Control and Prevention (CDC) and World Health Organization (WHO). The prevalent symptoms were assessed, as well. Results Based on the CDC’s criteria, 18.3, 28.8, and 25.8% of the children had short stature, low body weight, and low BMI, respectively. These measures were obtained as 10, 22.4, and 13.9% according to the WHO’s categorization respectively. Furthermore, the most common symptoms among the children were abdominal pain (56.5%), skeletal pain (28%), constipation (27.4%), and anemia (23.8%). Conclusion To sum up, the results clearly indicated that growth failure and low height, weight, and BMI were prevalent among the children with CD. Moreover, in addition to gastrointestinal symptoms, a considerable number of patients had skeletal pain and anemia.

Focal, Symptomatic Bone Marrow Lesions in Waldenström Macroglobulinaemia Characterised By Dense Infiltration with Lymphoplasmacytic Lymphoma: A Newly Defined Indication for Systemic Treatment

BACKGROUND Waldenström Macroglobulinaemia (WM) is an indolent, IgM-producing lymphoplasmacytic lymphoma (LPL) that infiltrates bone marrow (BM) and other tissues. Only symptomatic patients (with IgM-related complications, cytopenias, constitutional symptoms, bulky extramedullary disease) require treatment. Symptomatic BM lesions are not a recognised manifestation of WM and, if present, may raise the possibility of high-grade transformation. AIMS To characterise the clinical, radiological and histological features of focal bone marrow lesions (FBML) identified in WM patients with unrelenting pain in bones and/or joints, and outline implications for treatment. METHODS This study retrospectively reviewed investigations performed at the time of FBMLpresentation, including MRI of the site of pain, total body FDG-PET/CT, review of histology from BM trephine biopsy of the posterior superior iliac spine (PSIS) and CT-guided core biopsy of the identified FBML, and routine blood panels. RESULTS The 6 patients identified (Table 1) presented with localised skeletal pain at different stages of their WM disease course: 1 at initial WM diagnosis, 1 after 10 years of observation, 3 in remission following R-chemotherapy, and 1 during active treatment with R-chemotherapy. Median age at diagnosis of WM was 57 years (41-64 years). Median time from diagnosis to presentation with FBML was 28 months (0-120 months). MRI demonstrated well-defined areas of abnormal signal within the medullae of symptomatic bones and no evidence of cortical or trabecular bone involvement (Fig. 1A), with T1 hypointensity, diffusion restriction and mild STIR hyperintensity in excess of background BM changes. The FBML showed a predilection for the lower limbs (knees, ankles and/or feet involvement in 3 patients, 50%) and periarticular spaces (5 patients, 83%). They were CT-occult and only mildly FDG-avid. In all cases, core biopsies of the FBML showed heavy BM infiltration with diffuse interstitial infiltrate of small, mature lymphocytes (Fig. 1B, 1C) expressing CD20, CD79a and IgM; CD138 staining highlighted scattered interstitial plasma cells (<10%). There was no evidence of infarct or high-grade transformation. When compared to contemporaneous PSIS biopsies (Fig. 1D, 1E), all FBML demonstrated significantly higher proportions of lymphoid infiltration into the haematopoietic space (80-100% vs 0-80%, p=0.03), confirming FBML represent heavier disease infiltrationthan the background BM disease burden. On blood panels, LDH, ALP and platelet count were normal and Hb was >100g/L in all but the 1 patient with active WM (who had Hb 79g/L and ALP 149IU/L). There was no corresponding rise in paraprotein at the time of FBML presentation. In all cases, the detection of FBML prompted initiation/escalation of systemic treatment despite no evidence of progression using conventional criteria; this resulted in resolution of pain and disappearance of the T1 hypointense lesions on MRI in all cases. CONCLUSION Painful, LPL-dense, infiltrative lesions confined to the BM of affected appendicular bones are an unreported manifestation in WM. They are clinicopathologically different from the osteolytic lesions seen in myeloma and the painless, diffuse BM infiltration seen in low-grade lymphomas and can induce unrelenting focal skeletal pain. Based on our findings, such cases should be evaluated for focal bone marrow lesions; MRI is the preferred modality as lesions are CT-occult. They do not necessarily represent high-grade transformation. This small yet comprehensive series suggests they constitute a hitherto undescribed novel indication for systemic therapy irrespective of the general disease status. Disclosures D'Sa: Janssen:Honoraria, Research Funding;BeiGene:Honoraria, Research Funding;Sanofi:Honoraria.