Pure Red Cell Aplasia Associated with Monoclonal Gammopathy of Undetermined Significance and Literature Review.
BACKGROUND
Pure red cell aplasia (PRCA) is an uncommon disease which involves an almost complete absence of the erythroid lineage in bone marrow (BM) and causes severe anemia. Cases due to monoclonal gammopathy occurring in plasma cell disorder have been infrequently reported. Here we report a case of PRCA associated plasma cell disorder, especially monoclonal gammopathy of undetermined significance (MGUS).


METHODS
A 55-year-old male visited the ER due to general weakness. At his initial visit he exhibited severe anemia. Mild intravascular hemolysis was suspected. For anemia evaluation, BM examination was performed. In BM aspiration, almost no erythroid precursor cells were observed. Also, plasma cells were relatively elevated, at 7.2%. Serum electrophoresis and immunofixation revealed paraproteinemia of 5.1 g/L (IgG and lambda). No hypercalcemia, renal insufficiency or lytic bone lesions were found. This unusual case showed MGUS accompanied by PCRA. We were also able to assume the erythroid cell-specific restriction due to paraprotein, because we ruled out possible causes of PRCA.


RESULTS
We discovered several reported cases associated with plasma cell dyscrasia. However, most of these cases involved plasma cell myeloma, characterized by high immunoglobulin burden. Our case demonstrates that PRCA is also observed in cases with MGUS, where immunoglobulin burden is low.


CONCLUSIONS
It is not yet accurately known, what parts of erythroid precursors are targeted by M-protein nor what the mechanism is. Therefore, additional research into this matter is necessary.
Hyunjung
H
Gu
Woo-In
WI
Lee
You La
Y
Jeon
So Young
SY
Kang
Myeong Hee
MH
Kim
Tae Sung
TS
Park
D000740:Anemia / Q000209:etiology; D015415:Biomarkers / Q000097:blood; D001856:Bone Marrow Examination; D017707:Erythrocyte Transfusion; D015672:Erythroid Precursor Cells / Q000276:immunology; D006801:Humans; D008297:Male; D008875:Middle Aged; D008998:Monoclonal Gammopathy of Undetermined Significance / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D009101:Multiple Myeloma / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D009194:Myeloma Proteins / Q000276:immunology; D012010:Red-Cell Aplasia, Pure / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000628:therapy
D015415:Biomarkers; D009194:Myeloma Proteins; C085016:multiple myeloma M-proteins