Contributions
Abstract: PB2236
Type: Publication Only
Background
Light Chain Amyloidosis (AL) is a systemic disease which causes extracellular deposition of amyloid fibrils composed of immunoglobulin light chains secreted by monoclonal plasma cells. Splenic involvement is defined by functional hyposplenism identified by the presence of Howell-Jolly bodies in the red cells on peripheral blood smear in patients without splenectomy. Prevalence of hyposplenism in AL patients is 24%. Hyposplenism was identified in patients with AL and FX deficit. Splenomegaly is not a useful sign for the diagnosis of functional hyposplenism in amyloidosis. Hyposplenic patients had a worse survival compared to normosplenic amyloidosis patients, probably due to the extensive body burden of amyloid.
Aims
The aim was to study the presence of hyposplenism and it's association with pancytosis in light chain amyloidosis.
Methods
We studied the patients diagnosed with AL in the Hematology Department of Fundeni Clinical Institute, Bucharest, Romania, between 2007 and 2017. Functional hyposplenism was identified by the presence of Howell-Jolly bodies in the red cells on the peripheral blood smear. We evaluated CBC, age, sex, AL type, FX level and involved organs.
Results
In the last 10 years, there were diagnosed 124 patients with AL, 40 patients (32%) had Howell-Jolly bodies in the red cells and 13 patients (10%) had leukocytosis +/- thrombocytosis. Median age of patients with functional hyposplenism was 62 years, M:F ratio was 1:1 and 38% were κ AL and 62% λ AL. The association of leukocytosis and presence of Howell-Jolly bodies in the red cells on peripheral blood smear was present in 6 patients, of which 4 had cardiac and kidney involvement and 4 had liver and peripheral nervous system involvement. Hepatomegaly was seen in 4 of the 6 patients. The average FX level was 69% (min. 30% and max 110%), but without clinically significant bleeding, except amyloid vasculitis.
Case presentation. A 47 years old male diagnosed in 2014 with pancytosis (WBC 14 310/µL; PLT 906 000/µL, HB 19.4 g/dl; Ht 56.4%) and hepatomegaly. Bone marrow aspirate and biopsy were performed and he was diagnosed, in another Hematology Department, with chronic myeloproliferative disease for which he received 4 phlebotomies and hydroxyurea for 6 months. In October 2014 he is diagnosed with nephrotic syndrome and has a renal biopsy that shows amyloid deposits (kappa light chain). In November 2014 he was admitted to our Clinic and was diagnosed with kappa light chain amyloidosis with systemic involvement: kidney (nephrotic syndrome), liver (massive hepatomegaly, Stiffness of 75 KPa on Fibroscan), splenic (pancytosis, Howell-Jolly bodies in the red cells, splenomegaly). We started treatment with CyBorD (Cyclophosphamide, Bortezomib and Dexamethasone), but the patient wasn’t compliant and was lost from treatment and follow up for about 1 year. In this period, he developed severe kidney disease and started hemodialysis. He came back to our Clinic in November 2015 and received 7 cycles of CyBorD. Involved free light chain decreased from 519 mg/l to 44 mg/l, there was an impressive improvement in Fibroscan stiffness (75->13.8 KPa) and maintained a slight leukocytosis and thrombocytosis. He is without treatment since June 2016, in excellent clinical condition, normal blood count, but he is still on hemodialysis.
Conclusion
Amyloidosis with splenic involvement and secondary hyposplenism can be the cause of pancytosis and so it should be considered, especially if Howell-Jolly bodies in the red cells are present on the peripheral blood smear.
Session topic: 14. Myeloma and other monoclonal gammopathies - Clinical
Keyword(s): Amyloidosis, Spleen
Abstract: PB2236
Type: Publication Only
Background
Light Chain Amyloidosis (AL) is a systemic disease which causes extracellular deposition of amyloid fibrils composed of immunoglobulin light chains secreted by monoclonal plasma cells. Splenic involvement is defined by functional hyposplenism identified by the presence of Howell-Jolly bodies in the red cells on peripheral blood smear in patients without splenectomy. Prevalence of hyposplenism in AL patients is 24%. Hyposplenism was identified in patients with AL and FX deficit. Splenomegaly is not a useful sign for the diagnosis of functional hyposplenism in amyloidosis. Hyposplenic patients had a worse survival compared to normosplenic amyloidosis patients, probably due to the extensive body burden of amyloid.
Aims
The aim was to study the presence of hyposplenism and it's association with pancytosis in light chain amyloidosis.
Methods
We studied the patients diagnosed with AL in the Hematology Department of Fundeni Clinical Institute, Bucharest, Romania, between 2007 and 2017. Functional hyposplenism was identified by the presence of Howell-Jolly bodies in the red cells on the peripheral blood smear. We evaluated CBC, age, sex, AL type, FX level and involved organs.
Results
In the last 10 years, there were diagnosed 124 patients with AL, 40 patients (32%) had Howell-Jolly bodies in the red cells and 13 patients (10%) had leukocytosis +/- thrombocytosis. Median age of patients with functional hyposplenism was 62 years, M:F ratio was 1:1 and 38% were κ AL and 62% λ AL. The association of leukocytosis and presence of Howell-Jolly bodies in the red cells on peripheral blood smear was present in 6 patients, of which 4 had cardiac and kidney involvement and 4 had liver and peripheral nervous system involvement. Hepatomegaly was seen in 4 of the 6 patients. The average FX level was 69% (min. 30% and max 110%), but without clinically significant bleeding, except amyloid vasculitis.
Case presentation. A 47 years old male diagnosed in 2014 with pancytosis (WBC 14 310/µL; PLT 906 000/µL, HB 19.4 g/dl; Ht 56.4%) and hepatomegaly. Bone marrow aspirate and biopsy were performed and he was diagnosed, in another Hematology Department, with chronic myeloproliferative disease for which he received 4 phlebotomies and hydroxyurea for 6 months. In October 2014 he is diagnosed with nephrotic syndrome and has a renal biopsy that shows amyloid deposits (kappa light chain). In November 2014 he was admitted to our Clinic and was diagnosed with kappa light chain amyloidosis with systemic involvement: kidney (nephrotic syndrome), liver (massive hepatomegaly, Stiffness of 75 KPa on Fibroscan), splenic (pancytosis, Howell-Jolly bodies in the red cells, splenomegaly). We started treatment with CyBorD (Cyclophosphamide, Bortezomib and Dexamethasone), but the patient wasn’t compliant and was lost from treatment and follow up for about 1 year. In this period, he developed severe kidney disease and started hemodialysis. He came back to our Clinic in November 2015 and received 7 cycles of CyBorD. Involved free light chain decreased from 519 mg/l to 44 mg/l, there was an impressive improvement in Fibroscan stiffness (75->13.8 KPa) and maintained a slight leukocytosis and thrombocytosis. He is without treatment since June 2016, in excellent clinical condition, normal blood count, but he is still on hemodialysis.
Conclusion
Amyloidosis with splenic involvement and secondary hyposplenism can be the cause of pancytosis and so it should be considered, especially if Howell-Jolly bodies in the red cells are present on the peripheral blood smear.
Session topic: 14. Myeloma and other monoclonal gammopathies - Clinical
Keyword(s): Amyloidosis, Spleen