UNASSIGNED: given the lack of information about monoclonal gammopathies, our primary study outcome was to describe the epidemiological, clinical and biochemical profiles of monoclonal gammopathies in the Souss-Massa region, in southern Morocco.
UNASSIGNED: we conducted a retrospective study, by selecting only complete medical records. We used records of patients diagnosed with monoclonal gammopathy at the local oncology center during a period of over 10 years.
UNASSIGNED: one hundred and seventeen patients were included in the study, with a high male predominance (65%) and a male/female sex-ratio of 1.85. The average age of our study population was 61.44 (ET 14.54) years. Diagnoses (based on frequency) included: multiple myeloma 82.0% (n=96), solitary plasmacytoma 8.5% (n=10), monoclonal gammopathies of undetermined significance 2.6% (n=3), lymphoma 2.5% (n=3), secondary plasma cell leukaemia 1.7% (n=2), Waldenström´s disease 1.7%(n=2) and chronic lymphoid leukemia (n=1). The isotype distribution was as follows: IgG Kappa 33.7% (n=28), IgG lambda 21.7% (n=18), IgA Kappa 12.0% (n=10), IgA lambda 7.2% (n=6), IgM kappa 3.6% (n=3), and IgD lambda 2.4% (n=2). Biconal peak was reached in two cases, with a percentage of 2.4%.
UNASSIGNED: diagnostic delay was observed compared to international studies due to the unavailability of electrophoresis in the care structures.

UNASSIGNED: Hypergammaglobulinemic purpura of Waldenström is an uncommon disease, which presents mostly in women on the lower extremities. It is sometimes associated with underlying immune dysregulation. Sjögren syndrome is the most common association; however, rare occurrences of the self-resolving syndrome with lymphoma or myeloma have been reported.
UNASSIGNED: We describe an unusual and impressive presentation of hypergammaglobulinemic purpura of Waldenström in an elderly female patient with myeloma. Notably, the patient did not have any concurrent connective tissue diseases. Despite her florid presentation, her hypergammaglobulinemic purpura of Waldenström spontaneously resolved within a few days.
UNASSIGNED: Hypergammaglobulinemic purpura of Waldenström is a self-resolving but recurrent syndrome, which may be associated with autoimmune disorders or rarely myeloma. Early diagnosis of the syndrome may avoid unnecessary treatment interventions and should prompt screening for underlying diseases.

Waldenström macroglobulinemia (WM) is a rare lymphoproliferative disorder. Up to now, therapeutic choice was not influenced by the biological characteristics of the disease. Here, we will review how recent advances in biology in WM may affect therapy strategy.
Recently, WM has been described as a new oncogenic model. MyD88 mutation has been described as a key driver mutation and has functional consequences which could be targeted. Other mutations, such as CXCR4 or TP53, have been reported. These mutations are associated with different clinical presentation, prognosis, and treatment response. Mutational status may influence therapeutic choice in some patients but additional data are required. New targeted therapies are on development.

In a first series from India, we report 32 cases of lymphoplasmacytic lymphoma/Waldenström macroglobulinemia (LPL/WM) over 7 years. Here, we analyzed 32 patients with LPL/WM for MYD88 L265P mutation and correlated mutation staus with hematological and biochemical parameters and also with the International Prognostic Scoring System (ISSWM) and treatment response. Twenty-seven out of 32 cases of LPL/WM (84.3%) harbored the MYD88 L265P mutation. MYD88 wild-type WM was associated with a lower number of tumor cells (p<0.01) and older age (p=0.02) and a lower ISSWM score at presentation (p=0.03) as compared to mutated LPL/WM. On evaluation of response (n=23), 44.4% of patients with MYD88 mutated LPL/WM had progressive disease, whereas no patient in the MYD88 unmutated group changed their baseline status. We confirm the high frequency of MYD88 mutations in LPL/WM. Although the number of MYD88 wild-type cases was limited, our data indicate that MYD88 may represent an adverse prognostic marker for LPL/WM.