Multiple Myeloma Orthobullets

Multiple Myeloma Orthobullets - Multiple Myeloma (MM) is characterized by progressive proliferation of malignant plasmocytes, usually beginning in the bone marrow. MM may affect any organ, a total of 7-18% of patients with MM show extramedullary involvement at the time of diagnosis. Non-secretory multiple myeloma (NSMM) is a rare variant that includes 1 to 5% of all multiple myeloma cases. This disease is characterized by the absence of monoclonal gammopathy in serum and urinary electrophoresis. Our case report highlights the diagnostic challenges of NSMM cases with extensive extramular involvement in patients with young women who initially experience right shoulder pain and bilateral breast nodules. The skeleton survey shows several lytic bone lesions. The initial diagnosis is primary breast carcinoma with bone metastasis. There is no monoclonal gammopathy found in serum or urine electrophoresis. Bone marrow and breast biopsy show severe plasmocytosis. Diagnosis is delayed for one month due to the lack of clinical suspicion of multiple myeloma in young patients and a slight biochemical expression of non-secretory multiple myeloma types.

Multiple Myeloma (MM) is characterized by abnormal proliferation of plasma cells, usually originating from the bone marrow. The most common manifestation is bone involvement with the finding of a diffuse radiological picture of osteopenia or osteolytic bone lesions. Up to 7-18% of the MM cases were associated with extramedullary involvement (EM) at the time of diagnosis. IN MM Plasmocytoma may occur during the course of the disease or relapse. This can arise from any tissue, and its existence has been associated with more aggressive diseases. One of the diagnostic criteria of MM is the detection of monoclonal gammopathy in serum and/or urine electrophoresis. When it is not present, this condition is known as non-secretory multiple myeloma, making the diagnosis more difficult. This MM subtype represents about 1 to 4% of all MM cases.

Multiple Myeloma Orthobullets

Multiple Myeloma Orthobullets - A 31-year-old woman presented a three-week history of right shoulder pain associated with lethargy, palpitations and shortness of breath. Right shoulder pain is not preceded by trauma. Your past medical history is mediocre. Physical examination shows intense sensitivity in the right shoulder. However, there is no limit to the movement. He was pale, with a blood pressure of 110/50 mmHg and a heart rate of 98/minute. Some breast nodules that are tense, in motion, and not chest pain, are felt bilaterally. However, there is no axillary lymphadenopathy detected. There is bilateral proptosis that is more dominant on the right side. The rest of the physical exam is mediocre. Blood tests showed normocytic normocytic anemia: Hemoglobin levels measured 6.5 g/dL.

This shows some well-defined soft tissue masses that involve subcutaneous tissue, breast, paraspinal area, left renal pelvis and lungs. Both kidneys are in normal size and thickness of the renal parenchyma. There are extensive lytic bone lesions, some with soft tissue components. Photon emission computed tomography (SPECT) showed no evidence of abnormal radio-pharmaceutical absorption to suggest bone metastasis. The orbital CT, subsequently performed due to the worsening of the proptosis of the right eye, reveals the right extraconal mass. The temporomandibular joints, including the orbital CT, show multiple lytic lesions involving the two condylar heads. Based on these findings, a rare diagnosis of multiple myeloma with Extra-medular Plasmocytoma is considered.

Moreover, the histopathological analysis of breast nodules showed diffuse malignant infiltration by plasmocytes, which presented Mamarium Plasmacytoma. A bone marrow biopsy is performed and shows more than 80% of abnormal plasma cells. Thrombin biopsy shows total infiltration by plasmocytes showing plasma cell myeloma. The final diagnosis of non-secretory multiple myeloma (Durie-Salmon stage III) with Extramedullary disease is made. There is a one-month delay in diagnosis due to the lack of clinical suspicion for this unusual phenomenon and the scarcity of non-secretory multiple myeloma.

During the course of the disease, he suffered a pathological fracture of the right humerus. Its condition is complicated by hypercalcemia (3.78 mg/dL) and renal insufficiency (serum creatinine 239 micromoles/L), but normalized in a duration of 10 days after hydration and correction of sufficient hypercalcemia. It began with the use of intravenous bortezomib, cyclophosphamide, dexamethasone and three times intrathecal chemotherapy. All the in Plasmacytomas were completely resolved after the third cycle of chemotherapy and the trephine biopsy did not reveal excess plasma cells that presented a good chemotherapeutic response. He continued five more cycles of chemotherapy and planned autologous stem cell transplantation. During treatment, he suffered several episodes of neutropenic sepsis and hemorrhagic cystitis due to chemotherapy. Unfortunately, this disease fell one month after the eighth cycle of chemotherapy with several new medullary and extramular lesions. He developed several cranial nerve palsy and spinal cord compression. The results of lumbar puncture reveal plasma cells scattered in the cerebrospinal fluid. He finally gave up, 11 months after the initial presentation, due to the involvement of the aggressive cranial nervous system with leptomeningeal myelomatosis.

Multiple myeloma is rare in young patients. Patients under 40 years of age constitute 2.2% of all cases of multiple myeloma, while those younger than 30 years are only 0.3% of all cases.

Non-secretory multiple myeloma (NSMM) is a rare variant that represents 1 to 5% of all multiple myeloma cases. This disease is characterized by the absence of monoclonal gammopathy in serum and urine by immunofixation electrophoresis. NSMM is associated with greater survival, but these patients usually present a more advanced disease than classic myeloma during the initial presentation.

NSMM complicated by renal insufficiency is rare, given the absence of light chains in the urine. In this case, we consider several possible causes of renal disorders. There are no ongoing renal infections, episodes of hypotension or drug-related causes. Although renal biopsy is not performed, imaging exams do not show swollen kidneys, suggesting obstructive nephropathy or plasma cell infiltration. We believe that renal damage in these patients is caused by dehydration and hypercalcemia because it improves with hydration and normalization of calcium levels.

The MM disease is defined as the presence of malignant plasmocytes outside the bone marrow, both as soft tissue masses that spread from the bone or arise from the organs of MM. It is not uncommon to see intramedullary myeloma with extension of MM as a result of cortical erosion and sub Dissemination outside the Periosteo. However, the dissemination of malignant hematogenous plasma cells to MM regions is less common. This method of dissemination is responsible for 3.4% of cases at the time of diagnosis, 5% of recurrence and during the course of the disease. The MM disease may affect any organ and appear as subcutaneous nodules, renal mass, breast nodules, orbital mass, lung and pleural nodules. Imaging findings are often nonspecific and often mimic other disturbances.

In this case, the patient has extensive involvement of the medulla and the MM that affects the left kidney, both the breast, subcutaneous tissue, right orbit, lungs, paraspinal area and Lepto-meninges. Due to the extensive differential diagnosis, the clinical manifestations of this patient actually cause a diagnostic dilemma. Our first impression is primary breast carcinoma with extensive metastasis. However, breast carcinoma with advanced metastasis is mainly accompanied by regional or distant lymphadenopathy. In addition, Bone scintigraphy is expected to show extensive radiotherapic absorption or "superscan " appearance. On the other hand, there is no lymphadenopathy in our patients and bone scintigraphy is negative for bone metastasis.