ACQUIRED IRON-REFRACTORY IRON DEFICIENCY ANEMIA (ACQUIRED IRIDA): A TUMOR RELATED DISORDER.
(Abstract release date: 05/19/16)
EHA Library. Remacha A. 06/09/16; 135038; PB2138
Dr. Angel F Remacha
Contributions
Contributions
Abstract
Abstract: PB2138
Type: Publication Only
Background
Mutations in the TMPRSS6 (matriptase-2) gene are associated with severe iron-refractory iron deficiency anemia (IRIDA) resulting from an overexpression of hepcidin. Microcytic anemia is the hallmark of this disorder. A similar form of acquired microcytic anemia has been observed in several situations, including benign tumors (1) or malignant tumors (1), treatment with m-TOR inhibitors (3).
Aims
To evaluate the prevalence of this acquired form of microcytic anemia.
Methods
From the group of patients with anemia studied in our red cell pathology unit for a year those with microcytic anemia and biochemical data similar to IRIDA were clinically evaluated.
Results
A total of 3911 anemia patients were studied for a year, including 882 (22.5%) with microcytic anemia. Nine of these 882 patients (1% of microcytic anemia, and 0.2 % of total) showed a pattern similar to IRIDA. Main analytical data of these 9 patients are included the table below. The microcytic anemia showed mixed inflammatory (increased SR and SF) and iron deficiency (low SI and high sTfR) signs. In all cases the diagnosis of the microcytic anemia was at the onset of the disease, and in 8 out of the 9 was related to neoplasia, including: renal carcinoma (2 cases), carcinomatosis peritoneal due to bladder neoplasia, disseminated mesotelioma, bladder carcinoma and lung carcinoma, renal lymphoma, disseminated neoplasia of probable bladder origin, rectal adenocarcinoma with a perirenal mass. Six of them died. The remaining case was an HIV patient with pneumocystis jirovecii pneumonia, after pneumonia treatment anemia disappeared.
Conclusion
This microcytic anemia was rare, but it was related with malignant tumor with very poor prognosis, mainly involving kidney. Despite its rarity, this diagnosis should be considered owing to the poor prognosis. The term acquired IRIDA is proposed for this type of acquired microcytic anemia. 1. Weinstein DA, Roy CN, Fleming MD, Loda MF, Wolfsdorf JI, Andrews NC. Inappropriate expression of hepcidin is associated with iron refractory anemia: implications for the anemia of chronic disease. Blood 2002;100:3776-81.2. Kamai T, Tomosugi N, Abe H, Arai K, Yoshida KI. Increased serum hepcidin-25 level and increased tumor expression of hepcidin mRNA are associated with metastasis of renal cell carcinoma. BMC Cancer 2009, 9:2703. Kaplan B, Qazi Y, Wellen JR. Strategies for the management of adverse events associated with mTOR inhibitors. Transplant Rev (Orlando) 2014;28:126-33.
Session topic: E-poster
Keyword(s): Ferritin, Iron deficiency anemia, Renal cell carcinoma, Transferrin receptor
Type: Publication Only
Background
Mutations in the TMPRSS6 (matriptase-2) gene are associated with severe iron-refractory iron deficiency anemia (IRIDA) resulting from an overexpression of hepcidin. Microcytic anemia is the hallmark of this disorder. A similar form of acquired microcytic anemia has been observed in several situations, including benign tumors (1) or malignant tumors (1), treatment with m-TOR inhibitors (3).
Aims
To evaluate the prevalence of this acquired form of microcytic anemia.
Methods
From the group of patients with anemia studied in our red cell pathology unit for a year those with microcytic anemia and biochemical data similar to IRIDA were clinically evaluated.
Results
A total of 3911 anemia patients were studied for a year, including 882 (22.5%) with microcytic anemia. Nine of these 882 patients (1% of microcytic anemia, and 0.2 % of total) showed a pattern similar to IRIDA. Main analytical data of these 9 patients are included the table below. The microcytic anemia showed mixed inflammatory (increased SR and SF) and iron deficiency (low SI and high sTfR) signs. In all cases the diagnosis of the microcytic anemia was at the onset of the disease, and in 8 out of the 9 was related to neoplasia, including: renal carcinoma (2 cases), carcinomatosis peritoneal due to bladder neoplasia, disseminated mesotelioma, bladder carcinoma and lung carcinoma, renal lymphoma, disseminated neoplasia of probable bladder origin, rectal adenocarcinoma with a perirenal mass. Six of them died. The remaining case was an HIV patient with pneumocystis jirovecii pneumonia, after pneumonia treatment anemia disappeared.
Conclusion
This microcytic anemia was rare, but it was related with malignant tumor with very poor prognosis, mainly involving kidney. Despite its rarity, this diagnosis should be considered owing to the poor prognosis. The term acquired IRIDA is proposed for this type of acquired microcytic anemia. 1. Weinstein DA, Roy CN, Fleming MD, Loda MF, Wolfsdorf JI, Andrews NC. Inappropriate expression of hepcidin is associated with iron refractory anemia: implications for the anemia of chronic disease. Blood 2002;100:3776-81.2. Kamai T, Tomosugi N, Abe H, Arai K, Yoshida KI. Increased serum hepcidin-25 level and increased tumor expression of hepcidin mRNA are associated with metastasis of renal cell carcinoma. BMC Cancer 2009, 9:2703. Kaplan B, Qazi Y, Wellen JR. Strategies for the management of adverse events associated with mTOR inhibitors. Transplant Rev (Orlando) 2014;28:126-33.
Session topic: E-poster
Keyword(s): Ferritin, Iron deficiency anemia, Renal cell carcinoma, Transferrin receptor
Abstract: PB2138
Type: Publication Only
Background
Mutations in the TMPRSS6 (matriptase-2) gene are associated with severe iron-refractory iron deficiency anemia (IRIDA) resulting from an overexpression of hepcidin. Microcytic anemia is the hallmark of this disorder. A similar form of acquired microcytic anemia has been observed in several situations, including benign tumors (1) or malignant tumors (1), treatment with m-TOR inhibitors (3).
Aims
To evaluate the prevalence of this acquired form of microcytic anemia.
Methods
From the group of patients with anemia studied in our red cell pathology unit for a year those with microcytic anemia and biochemical data similar to IRIDA were clinically evaluated.
Results
A total of 3911 anemia patients were studied for a year, including 882 (22.5%) with microcytic anemia. Nine of these 882 patients (1% of microcytic anemia, and 0.2 % of total) showed a pattern similar to IRIDA. Main analytical data of these 9 patients are included the table below. The microcytic anemia showed mixed inflammatory (increased SR and SF) and iron deficiency (low SI and high sTfR) signs. In all cases the diagnosis of the microcytic anemia was at the onset of the disease, and in 8 out of the 9 was related to neoplasia, including: renal carcinoma (2 cases), carcinomatosis peritoneal due to bladder neoplasia, disseminated mesotelioma, bladder carcinoma and lung carcinoma, renal lymphoma, disseminated neoplasia of probable bladder origin, rectal adenocarcinoma with a perirenal mass. Six of them died. The remaining case was an HIV patient with pneumocystis jirovecii pneumonia, after pneumonia treatment anemia disappeared.
Conclusion
This microcytic anemia was rare, but it was related with malignant tumor with very poor prognosis, mainly involving kidney. Despite its rarity, this diagnosis should be considered owing to the poor prognosis. The term acquired IRIDA is proposed for this type of acquired microcytic anemia. 1. Weinstein DA, Roy CN, Fleming MD, Loda MF, Wolfsdorf JI, Andrews NC. Inappropriate expression of hepcidin is associated with iron refractory anemia: implications for the anemia of chronic disease. Blood 2002;100:3776-81.2. Kamai T, Tomosugi N, Abe H, Arai K, Yoshida KI. Increased serum hepcidin-25 level and increased tumor expression of hepcidin mRNA are associated with metastasis of renal cell carcinoma. BMC Cancer 2009, 9:2703. Kaplan B, Qazi Y, Wellen JR. Strategies for the management of adverse events associated with mTOR inhibitors. Transplant Rev (Orlando) 2014;28:126-33.
Session topic: E-poster
Keyword(s): Ferritin, Iron deficiency anemia, Renal cell carcinoma, Transferrin receptor
Type: Publication Only
Background
Mutations in the TMPRSS6 (matriptase-2) gene are associated with severe iron-refractory iron deficiency anemia (IRIDA) resulting from an overexpression of hepcidin. Microcytic anemia is the hallmark of this disorder. A similar form of acquired microcytic anemia has been observed in several situations, including benign tumors (1) or malignant tumors (1), treatment with m-TOR inhibitors (3).
Aims
To evaluate the prevalence of this acquired form of microcytic anemia.
Methods
From the group of patients with anemia studied in our red cell pathology unit for a year those with microcytic anemia and biochemical data similar to IRIDA were clinically evaluated.
Results
A total of 3911 anemia patients were studied for a year, including 882 (22.5%) with microcytic anemia. Nine of these 882 patients (1% of microcytic anemia, and 0.2 % of total) showed a pattern similar to IRIDA. Main analytical data of these 9 patients are included the table below. The microcytic anemia showed mixed inflammatory (increased SR and SF) and iron deficiency (low SI and high sTfR) signs. In all cases the diagnosis of the microcytic anemia was at the onset of the disease, and in 8 out of the 9 was related to neoplasia, including: renal carcinoma (2 cases), carcinomatosis peritoneal due to bladder neoplasia, disseminated mesotelioma, bladder carcinoma and lung carcinoma, renal lymphoma, disseminated neoplasia of probable bladder origin, rectal adenocarcinoma with a perirenal mass. Six of them died. The remaining case was an HIV patient with pneumocystis jirovecii pneumonia, after pneumonia treatment anemia disappeared.
Conclusion
This microcytic anemia was rare, but it was related with malignant tumor with very poor prognosis, mainly involving kidney. Despite its rarity, this diagnosis should be considered owing to the poor prognosis. The term acquired IRIDA is proposed for this type of acquired microcytic anemia. 1. Weinstein DA, Roy CN, Fleming MD, Loda MF, Wolfsdorf JI, Andrews NC. Inappropriate expression of hepcidin is associated with iron refractory anemia: implications for the anemia of chronic disease. Blood 2002;100:3776-81.2. Kamai T, Tomosugi N, Abe H, Arai K, Yoshida KI. Increased serum hepcidin-25 level and increased tumor expression of hepcidin mRNA are associated with metastasis of renal cell carcinoma. BMC Cancer 2009, 9:2703. Kaplan B, Qazi Y, Wellen JR. Strategies for the management of adverse events associated with mTOR inhibitors. Transplant Rev (Orlando) 2014;28:126-33.
Session topic: E-poster
Keyword(s): Ferritin, Iron deficiency anemia, Renal cell carcinoma, Transferrin receptor
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