Clinical characteristics and diagnosis of a rare case of systemic AL amyloidosis: a descriptive study

Abstract

Systemic amyloidosis is a rare disease involving multiple organs. It is difficult to establish diagnosis as the symptoms is diverse and non-specific. And without specific therapy the prognosis is very poor. We analyzed detailed clinical and laboratorial data of a 53-year-old male patient. The characteristic features included refractory pleural effusion, extraordinary hepatomegaly and cardiac failure. The illness lasted 9 months and therapy period spanned 4 months. Fine needle biopsy of liver, lung, heart, pancreas and kidney was performed. Immunohistochemistry, immunofluorescence, Congo staining and hematoxylin and eosin staining were performed. All specimens were stained pink with haematoxylin and eosin staining. Amorphous deposits of eosinophilic material were visible within the Congo red dye stained liver tissue whereas under cross-polarized light pathognomonic apple-green birefringence of amyloid deposits was visible. At last systemic AL amyloidosis diagnosis was confirmed. The report showed an unusual AL amyloidosis case in detail which would be helpful for physician in clinical work.

Keywords: amyloidosis; hepatomegaly.

Figure 1. Imaging findings of lung, liver and heart

Chest CT showed inflammation and pleural effusion with right basal lobe atelectasis. (A) Abdominal ultrasonography indicated swollen liver (B). Echocardiography demonstrated left ventricular wall thickness was increased (C).

Figure 2. Bone marrow cytology examination

The percentage of plasma cell was within ranges (3.25%) and eosinophil granulocytes were easily noted.

Figure 3. Hematoxylin and eosin staining of multiple organs

There is amorphous extracellular material persent in sorts of tissue from liver (A), kidney (B), lung (C), heart (D) and pancreas (E). (magnification ×20).

Figure 4. Histological findings on liver tissue

Congo red staining of tissue biopsy, the gold standard for confirming amyloid deposition, a pink-orange color under lightmicroscope (A) and typical apple green briefringence under cross-polarised light (B). Imminohistochemical staining (C) by antibodies to serum amyloid P (SAP). Immunofluorescence analysis (D) showed bright green fluorescence using the novel luminescent conjugated polymer pentameric formicthiophene acetic acid (pFTAA). (magnification ×20).