Abstract
Introduction: Systemic AL amyloidosis is defined as extracellular deposition of insoluble immunoglobulin light chains, amyloid fibril protein AL, in at least one vital organ. Here we report a case of systemic AL amyloidosis presenting as progressive hepatic failure with coagulopathy.
Highlights
Systemic AL amyloidosis is defined as extracellular deposition of insoluble immunoglobulin light chains, amyloid fibril protein AL, in at least one vital organ [1]
We report a very rare case of systemic AL amyloidosis presenting as progressive hepatic failure with coagulopathy
Kappa and lambda in situ hybridization (ISH) was performed and showed predominant kappa expression in bone marrow. This patient had systemic AL amyloidosis causing severe progressive hepatic failure with coagulopathy due to underlying plasma cell dyscrasia in bone marrow, which was diagnosed by postmortem examination
Summary
Systemic AL amyloidosis is defined as extracellular deposition of insoluble immunoglobulin light chains, amyloid fibril protein AL, in at least one vital organ [1]. The patient was a 62-year-old African American female with past medical history of asthma and hypertension She presented at the emergency room with complaints of jaundice, easy bruising, fatigue, and weight loss of three months. Renal function was abnormal with slightly increased creatinine and urine protein was not checked (Table 1) She had profound anemia, hypoalbuminemia and severe coagulopathy with PT > 100 sec, partial thromboplastin time (PTT) > 150 sec, and platelet of 47 K/uL (Table 1). Kappa and lambda in situ hybridization (ISH) was performed and showed predominant kappa expression in bone marrow This patient had systemic AL amyloidosis causing severe progressive hepatic failure with coagulopathy due to underlying plasma cell dyscrasia in bone marrow, which was diagnosed by postmortem examination
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