Among 186 patients with multiple myeloma (MM), five women were diagnosed as having MM without M-component in serum and or urine at the diagnosis and along the evolution. Bone marrow plasmacytosis at greater than 30% was found in all patients and bone x-rays showed lytic lesions in all but one case, osteoporosis in all, and pathologic fractures in two. Serum electrophoresis showed a striking hypogammaglobulinemia in all, and polyclonal immunoglobulin levels were markedly reduced. The immunofluorescence of plasma cells in bone marrow was positive for monoclonal light chain polypeptides in four patients, and the ultrastructure showed mature plasmocytes with a wide rough endoplasmic reticulum (RER) and an intact Golgi apparatus. In three patients, therapy with melphalan plus prednisone was started. The remaining two were treated with an M-2 protocol. Death was an early event in two patients; the response was good in the remaining patients, without differences regarding secretory MM. Despite some reports stressing an unfavourable prognosis in MM without M-component, in our series it is roughly the same as in MM with secretion.

Nonsecretory multiple myeloma.

The clinical features of 13 patients with non-secretory myeloma from a series of 172 consecutive multiple myelomas are presented. The non-secretors survived significantly longer that the secretors, median 46 months versus 21 months (p less than 0.01). Non-secretory myeloma was associated with a higher incidence of neurological presentation, minimal lytic bone disease, a lower median percentage of plasma cells in the marrow and a lower incidence of hypogammaglobulinaemia. The median survival of the non-secretors with minimal lytic bone lesions was 74 months compared to 21 months for those with extensive bone disease. The superior survival of non-secretors is thus thought to be due to earlier presentation possibly as a result of a tendency to form symptomatic local tumours. Retrospective immunoperoxidase staining of stored tissue was performed in nine cases. In only one of these was a monoclonal immunoglobulin not detected indicating that true 'non-production' is rare. In four patients who produced a biochemically detectable paraprotein during the course of their disease and in whom immunoperoxidase data was available the immunoglobulin was of the same type. Immunoperoxidase staining may help in the initial diagnosis of non-secretory myeloma but did not contribute any prognostic information.

Non-secretory multiple myeloma: a report of 13 cases with a review of the literature.

A case of non-secretory multiple myeloma presenting as primary plasma cell leukaemia in a 65 year old woman is presented. Bone pain was the initial clinical manifestation. Laboratory analysis showed 20% of circulating immature plasma cells. Despite the presence of osteolytic lesions, no M-component could be demonstrated in serum protein electrophoresis, and serum and urine immunoelectrophoresis. Bone marrow aspirate demonstrated an 83% infiltration of plasma cells showing various degrees of immaturity. Immunofluorescence with monoclonal antisera demonstrated intracytoplasmic kappa light chains in a high percentage of plasma cells. Immature plasma cells without cellular capacity to synthesize and excrete complete immunoglobulins could be more aggressive, leading to an initial leukaemic process. Previous work regarding possible pathogenetic mechanisms, clinical and laboratory features, and response to treatment of this extremely rare association are reviewed.

https://pmj.bmj.com/content/postgradmedj/68/800/470.full.pdf

Non-secretory multiple myeloma presenting as primary plasma cell leukaemia.

Antiplatelet agents for the treatment of thrombotic thrombocytopenic purpura

Studies on the ultrastructure of tumor cells of 22 multiple myeloma patients revealed hitherto unreported observations concerning the Golgi apparatus and intermediate-size filaments. Various abnormalities of the Golgi apparatus including displacements of the Golgi stacks, dilatations of cisternae, and massive accumulations of vesicles as well as vacuoles in the Golgi region were found in myeloma cells of 11 patients.



Intermediate-size filaments forming a fine intracytoplasmic network were apparent in myeloma cells of all 22 patients. In five patients, in addition to this architecture, intermediate-size filaments were gathered into thick bundles which were primarily but not exclusively located in the perinuclear region.

Ultrastructural studies of myeloma cells: observations concerning the Golgi apparatus and intermediate-size filaments.

. A well documented case of non-secretory myelomatosis with extensive skeletal lesions has been studied by immunohistologic techniques, electron microscopy, various electrophoretic procedures and different studies of serum and urine. In addition, the various lymphocyte populations have been studied by immunofluorescent techniques, rosette formation and stimulation with PHA and PWM mitogens. The ultrastructural studies revealed a well preserved structure of most of the plasma cells, often with a prominent nucleolus. The Golgi apparatus (GOL) was often large and in some cells there appeared to be a striking development of non-granular (smooth) endoplasmic reticulum in association with the GOL. Various non-crystalline inclusions were seen outside the cisternae of the endoplasmic reticulum. The cells contained large amounts of immunoglobulins of the IgG (kappa) class. No paraprotein was present in serum. Normal amounts of serum Immunoglobulins were detected. No abnormalities could be demonstrated in the B or T cell system. It is concluded that the defect must be localized to the plasma cells themselves and not to the precursor cells of the B system.

"Non-secretory" plasma cell dyscrasia with normal serum immunoglobulins.

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"Non-secretory" plasma cell dyscrasia with normal serum immunoglobulins.