A 59 year old Iraqi women without past medical history, presented with pallor , easy fatigability and marked weight loss. At presentation here physical examination revealed anemia, no lymphadenopathy, no splenomegaly and huge hepatomegaly (liver span 20 cm) crossing to left hypochondrium. Her peripheral blood indices were as follows: white blood cell [WBC] 10 ×109/L, neutrophilic granulocyte 61%, lymphocyte 28%, hemoglobin 7.2 g/dl, platelet 301×109/L with myelocytes 2% and leukoerythroblastic blood picture. Biochemistry showed normal liver transaminase enzymes with high alkaline phosphatase 284.6 U/L (normal values 35-104 U/L), normal serum calcium, normal serum LDH level and normal renal function test. Serum protein electrophoresis and immunofixation electrophoresis showed restriction band of IgG kappa type.

Ultrasound of abdomen showed only huge hepatomegaly 24 cm, ECG and echocardiogram were normal. The Computed tomography (CT) / PET scan showed hepatomegaly with no focal pathological lesion or hyper metabolism, no metabolically active skeletal lesion and no significant abnormal metabolically active disease in the whole-body survey.

Bone marrow smears was normocellular with 10% abnormal plasma cells (binuclear and few flaming cells) (Fig. 1), multiple cloud-like, fluffy texture of the deposits (Fig. 2). Bone marrow biopsy showed normocellular marrow with interstitial amorphous pinkish deposition and interstitial infiltration by plasma cells (Fig. 3) giving a suggestion of bone marrow involvement by amyloidosis which was confirmed by positive Congo red stain (not shown by figure). Bone marrow aspirate flow cytometry revealed distinct cell cluster in the dim CD45 region comprising 7% of all gated events with the following phenotype: CD38+, CD138+, CD200+, CD117+, CD4–,CD56+, CD19+, CD20–, and  inconclusive light chain restriction.

The diagnosis of AL amyloidosis requires (1) demonstration of amyloid in tissue and (2) demonstration of a plasma cell dyscrasia and both of them were confirmed in this case so the final diagnosis was primary light chain (AL) amyloidosis which is the most common form of systemic amyloidosis and is associated with an underlying plasma cell dyscrasia.