Multiple myeloma with amyloidosis

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Multiple myeloma with amyloidosis is a rare condition characterized by light chains of immunoglobulins insoluble deposit and the aggregation of fibrils of amyloid in different tissues, causing progressive organ dysfunction and death.

The progression of the amyloidosis to a Multiple Myeloma is very uncommon, probably associated with short survival of patients with this diagnosis. On the contrary considered that you between 10-15% of patients with Multiple Myeloma develop Amyloidosis during its evolution, favoured by the development of new drugs for the management of this malignant hematologic, which have facilitated an increase in the survival of patients.

The clinical presentation of Amyloidosis is variable, depending on the extent of the deposits, the affected tissues, being very frequent systemic involvement, that the clinical manifestations of Multiple Myeloma-associated bring with them a very variable clinical picture, which sometimes makes it difficult to consider this diagnosis based on clinical evidence.Multiple myeloma with amyloidosis

In the clinical case presented to the debut of the disease were not characteristic of Multiple Myeloma laboratory results and clinical elements and that lacked specificity to make a clinical diagnosis based on the evidence, the curve electrophoretic spiked Polyclonal with fusion of beta and gamma, increase in the Alpha 1 and alpha 2 globulins features of chronic inflammatory processes, but with evidence of response to chemotherapy indicated.

Gastrointestinal clinical symptoms are uncommon in amyloidosis, and are the result of the direct involvement of the gastrointestinal tract at any level or the deposition of amyloid in the autonomic nervous system.

The appearance of deposits of amyloid in the liver is one of the most frequent signs of this disease, in the examination of the abdomen is usually noted the presence of hepatomegaly in one third of patients, maintaining a normal liver function up to the final stage of the disease, where the patient can die victim of a hepatic coma.

We should point out that in this patient predominated refractory dyspeptic symptoms to specific Therapeutics indicated, together with the progressive hepatomegaly despite hematological remission of multiple myeloma, constituting the presentation of this systemic disease clinic.

61% of Amyloidosis at the clinical elements of cardiovascular involvement, mainly caused by a restrictive infiltrative cardiomyopathy, due to the deposit of this substance between myocytes, cushioning the electrical potentials originating in the heart muscle and resulting in a decrease in ventricular compliance by ventricular stiffness (stiff heart) that explains the emergence of electrocardiographic abnormalities in this patient, as the micro-electrical voltage, associated with the increase in the thickness of the ventricular wall of the heart is known with the name of dissociation or ECO/ECG missmatc, the presence of a left anterior fascicular block and the beheading of the wave R v1 to V3.

The presence of this last electrocardiogram alteration is considered to be a patent of pseudoinfarto in the electrocardiogram and is related to the damping of electric potentials by woven amyloid.

Another element of interest in the case that concerns us, constituted what alterations in the Echocardiogram, as the increase in the thickness of the walls of the heart and the texture, features that are known with the name of sparkling oimagen in glass grinding, associated with hypertrophy of interventricular septum, this alteration that set an index forecast of life to the patient.

Patients who have an equal or greater than 15 mm interventricular septum are considered a median survival of less than 6 months compared with an average of two years for those who have an interventricular septum thickness equal to or less than 12 mm.

It should be noted that this patient had an 18 mm septum and he died seven months after the re-evaluation, with direct cause of death of acute left ventricular failure.

The kidney is the organ that most often affects (90% of cases), initially made it in the form of minimal proteinuria which then it acquires character nephrotic, slow, but progressively, it leads to kidney failure, making it one of the most common causes of death.

It remained elevated erythrocyte sedimentation rate values despite hematological remission of the disease, once culminated Induction chemotherapy, which we consider as the amyloid substance, is rich in glicosaminoglican, substance that may have fibrinogenesicas properties.


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