Unusual intracellular and extracellular crystal inclusions in light chain multiple myeloma.
نویسندگان
چکیده
Correspondence: Salvatore Casciaro, M.D., II Divisione di Medicina, Ospedale S. Martino, largo Rosanna Benzi, 16132 Genoa, Italy. Phone: international +39-010-5552392 – Fax: international +39-0105556661. Herein we report the light microscopy observation of intracellular and interstitial crystalloid deposits found in the bone marrow of two patients with light chain multiple myeloma (MM). The first patient was a 65-year old female referred to a nephrology unit because of acute renal failure, severe anemia (Hb 6.3 g/dL), and thrombocytopenia (Plt 92 109/L). Electrophoresis showed a marked reduction of total proteins, hypogammaglobulinemia and a monoclonal component ( light chain) in both serum and urine samples. Severe diffuse osteoporosis and multiple vertebral collapses were present. The second patient was a 74-year old female with IgG MM, already treated with melphalan-prednisone. She came to our observation because of severe anemia, thrombocytopenia, multiple osteolytic lesions and a nephrotic syndrome with excretion of chains. The two patients were partially responsive to chemotherapy (VAD and CTX-DEX) and survived 4 and 5 months. In both patients bone marrow specimens revealed extensive (80%) infiltration by immature and atypical plasma cells. The histologic pattern was characterized by the presence of May-Grünwald-Giemsa negative rods and needle-shaped inclusions (Figure 1), both in the plasma cell cytoplasm and in the intercellular spaces (Figure 2). Aggregates of these rods were also detected in the cytoplasm of macrophages (Figure 3). Additional investigations of these inclusions (i.e. electron microscopy and immunohistochemical characterization) could not be performed. In the first patient, periumbilical fat tissue was positive by Congo red staining, whereas marrow inclusions were negative. In the same patient, marrow aspiration after therapy showed a marked reduction of both plasma cells and inclusions (Figure 3). It is feasible, however, that the crystal inclusions may consist of monoclonal proteins formed and released by malignant plasma cells and subsequently phagocytosed by macrophages. Although in MM patients cytoplasmic and nuclear crystal inclusions have been already reported,1-3 our observation is of interest as it demonstrates the presFigure 1. Bone marrow smear stained by May-GrünwaldGiemsa (MGG) showing diffuse crystalloid deposits in light chain MM ( 40).
منابع مشابه
Intracellular and extracellular rhomboid shaped crystalline inclusions in a case of IgG lambda restricted plasma cell myeloma: a case report and review of the literature
The presence of crystalline inclusions in plasma cell myeloma is a rare phenomenon and cases have been reported with rod, needle, and rectangular shaped crystals. Here, we present a case of IgG lambda restricted plasma cell myeloma with rhomboid shaped intracellular crystalline inclusions and extracellular crystal depositions in the bone marrow. Since rhomboid crystal depositions can be seen in...
متن کاملEvidence for intracellular amyloid formation in myeloma.
Evidence for intracellular formation of amyloid fibrils in a patient with kappa light-chain myeloma is described. Amyloid fibrils were seen as intracytoplasmic inclusions within plasma cells, histiocytes, renal tubule cells, and possibly in hepatocytes. Extracellular amyloid was also present. The electron microscopic studies suggest that amyloid fibrils may form in the Golgi apparatus and withi...
متن کاملExtracellular and intracellular crystal deposition in plasma cell myeloma.
A 68-year-old man presented with pain and malaise. Laboratory studies showed pancytopenia, hypercalcemia, increased creatinine, and proteinuria. Serum protein electrophoresis revealed an M-spike (0.2 g/dL) and immunofixation confirmed l-light chain. Bone marrow showed sheets of l-light chain restricted plasma cells with readily identifiable extracellular hexagonal crystals on aspirate and biops...
متن کاملSerum Free Light-chain Assay for the Diagnosis, Management, and Prognosis of Multiple Myeloma
In recent decades, new serum biomarkers have been developed for routine laboratory practice, such as assaying serum free light chains and more recently, assaying immunoglobulin heavy and light chain isotypes (Hevylite). In this work, we highlight the interest of new biomarkers (Hevylite Test) in the management of monoclonal gammopathies because of the technical advantages it confers and the se...
متن کاملMultiple myeloma with intramedullary masses of M-component.
The bone marrow of a patient with light chain myeloma and amyloidosis was substantially infiltrated with basophilic globular particles. The globules, which were confined to the bone marrow, ranged in size from tiny intracytoplasmic inclusions to large (100 mu) extracellular particles. Both the intra- and extracellular globules stained with fluorescent antibody directed against the light chain (...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Haematologica
دوره 84 11 شماره
صفحات -
تاریخ انتشار 1999