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LETTER TO EDITOR Table of Contents   
Year : 2008  |  Volume : 51  |  Issue : 4  |  Page : 566-568
Infiltrative cardiomyopathy due to AL amyloidosis


1 Department of Pathology, Dr. Ram Manohar Lohia Hospital, New Delhi 110 001, India
2 Department of Medicine, Dr. Ram Manohar Lohia Hospital, New Delhi 110 001, India

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How to cite this article:
Batrani M, Marwah S, Buxi G, Sharma SC. Infiltrative cardiomyopathy due to AL amyloidosis. Indian J Pathol Microbiol 2008;51:566-8

How to cite this URL:
Batrani M, Marwah S, Buxi G, Sharma SC. Infiltrative cardiomyopathy due to AL amyloidosis. Indian J Pathol Microbiol [serial online] 2008 [cited 2020 Oct 1];51:566-8. Available from: http://www.ijpmonline.org/text.asp?2008/51/4/566/43767


Sir,

Primary systemic (AL type) amyloidosis is a light-chain immunoglobulin derived disorder. [1] It may or may not be associated with multiple myeloma. [1] Cardiac symptoms may be the first and only manifestation of the underlying amyloid deposition. [2]

A 50-year-old male patient presented with breathlessness on exertion for 1 month and bilateral swelling in the foot for 10 days. He had no history of hypertension, diabetes mellitus, or heart disease. On general physical examination, he had ascitis and bilateral pedal edema.

Hematological investigations showed hemoglobin levels of 12 g/dl, a total leukocyte count of 11.0 10 9 /L and a platelet count of 170 10 9 /L. A peripheral blood smear showed normocytic normochromic RBCs with rouleaux formation, a differential leukocyte count of neutrophils 76%, lymphocytes 20%, eosinophils 3% and monocyte 1%. Erythrocyte sedimentation rate (ESR) was 110 mm in the first hour by Westergren's method. Routine biochemistry investigations i.e., liver function tests, renal function tests and serum electrolytes including sodium, potassium and calcium were within normal limits. An electrocardiogram showed diffuse low voltage QRS complexes [Figure 1]. A 2D echocardiography revealed atrial septal hypertrophy and a 'granular sparkling' appearance of myocardium. A provisional diagnosis of congestive heart failure (CHF) due to infiltrative cadiomyopathy (possibly due to amyloidosis) was made.

An abdominal fat pad aspirate demonstrated Congo red positive deposits of amyloid [Figure 2A]. A bone marrow aspirate examination revealed 90% plasma cells. Few plasmablasts and multinucleate forms were also seen [Figure 2B]. Serum protein electrophoresis showed an 'M' spike. The final diagnosis was given as multiple myeloma with amyloidosis with infiltrative cardiomyopathy. The patient died 2 months after the diagnosis.

Primary AL amyloidosis is the most common form of systemic amyloidosis. [1] The classification of AL amyloidosis patients with myeloma and without myeloma is somewhat arbitrary and significant overlap exists. The clinical course is dominated by amyloidosis and it is rare to find the presence of myeloma related manifestations (lytic lesions, pathological fractures, anemia and myeloma cast nephropathy) in the presence of amyloidosis. [1] The percentage of bone marrow plasma cells is useful in making the distinction between myeloma-associated AL amyloid and AL amyloid without myeloma. An arbitrary threshold of 30% plasma cells is used to fulfill the criteria of multiple myeloma-associated amyloid, if no other clinical features of multiple myeloma are present. [1]

Cardiac manifestations are the most common clinical manifestation of AL amyloidosis. [1] Cardiac amyloidosis should be considered in any patient with CHF or cardiomyopathy in the absence of ischemic or valvular heart disease. [3] The diagnosis of cardiac amyloidosis requires a clinical suspicion based on a low voltage electrocardiogram coupled with echocardiographic findings of 'granular sparkling' myocardium, which is quite specific for amyloidosis. [2],[4] However, the confirmation of amyloidosis requires some form of tissue biopsy and demonstration of amyloid. [2],[4],[5] Subcutaneous fat pad aspiration with a reported sensitivity of 84% to 88% [4] is the preferred technique because of ease and simplicity of the procedure. [3] AL cardiac amyloidosis has the worst prognosis with a median survival of 1 to 2 years (4 months with features of heart failure). [4],[5] The heart is prognostically the most important organ determining the outcome of AL amyloidosis. [1] Death occurs most commonly as a result of progressive CHF or sudden death from arrhythmia. [4]


   Acknowledgement Top


We thank Dr. Rajbala Yadav and Dr. N.K. Chaturvedi for their support and guidance during the preparation of this manuscript.

 
   References Top

1.Gertz MA, Lacy MQ, Dispenzieri A. Immunolglobulin light chain amyloidosis (primary amyloidosis). In: Greer JP, Foerster J, Luhens JN, Rodgers GM, Paraskevas F, Glader B, editors. Wintrobe's clinical haematology. 11 th ed. Philadelphia: Lippincott; 2004. p. 2637-66.  Back to cited text no. 1    
2.Maredia N, Ray SG. Cardiac amyloidosis. Clin Med 2005;5:504-9.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Roy A, Roy V. Primary systemic amyloidosis: Early diagnosis and therapy can improve survival rates and quality of life. Postgrad Med 2006;119:93-9.  Back to cited text no. 3    
4.Shah KB, Inoue Y, Mehra MR. Amyloidosis and the heart: A comprehensive review. Arch Intern Med 2006;166:105-13.  Back to cited text no. 4    
5.Kothari SS, Ramakrishnan S, Bahl VK. Cardiac amyloidosis: An update. Indian Heart J 2004;56:197-203.  Back to cited text no. 5  [PUBMED]  

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Correspondence Address:
Meenakshi Batrani
Department of Pathology, Dr. Ram Manohar Lohia Hospital, New Delhi 110 001
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.43767

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  [Figure 1], [Figure 2A], [Figure 2B]



 

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