Contributions
Abstract: PB1841
Type: Publication Only
Background
Acquired pure red cell aplasia (PRCA) is a rare condition and is known to be associated with thymomas. Other associations have been described such as drugs, autoimmune conditions, malignancies and ABO incompatible stem cell transplants.
Aims
Here, we describe an unusual case of PRCA which was associated with both a thymoma and parvovirus infection. As part of the work-up for her thymoma, she was also found to have hypogammaglobulinaemia (Good's syndrome) and positive anti-acetylcholine receptor antibodies without clinical evidence of myasthenia gravis.
Methods
A 45-year-old lady presented with symptomatic anaemia to the haematology clinic. She had no past medical history of note prior to this consultation. She presented with symptoms of a viral illness at another hospital a few weeks prior and was found to have anaemia requiring transfusion.
On examination, she was well with mild pallor. The rest of her physical examination was unremarkable with no lymphadenopathy or hepatosplenomegaly. Her heart rate was 87 beats per minute (bpm) , blood pressure was 100/ 53 mmHg and her oxygen saturation was 100% on room air. Initial investigations revealed a normochromic, normocytic anaemia with a haemoglobin level of 8.3g/dL. Her white blood cell count (WBC) was 4.23 X 109/L and absolute neutrophil count (ANC) of 2.25 X 109/L. Her platelet count was normal. There was also reticulocytopenia with an absolute reticulocyte count of 5.80 X 109/L. The peripheral blood film showed a normochromic, normocytic anaemia with no other abnormalities seen.
A bone marrow examination was performed and revealed an absence of erythropoiesis with preserved granulopoetic and megakaryopoietic activity. Parvovirus PCR was positive as well from the bone marrow sample. A chest computed tomography (CT) demonstrated an anterior mediastinal mass (Figure 1). Given the CT findings, conditions associated with a thymoma was screened for and she was found to have a positive anti-acetylcholine receptor antibody titre of 1.76 nmol/L . Flow cytometry of the bone marrow showed B cell aplasia and the possibility of Good’s Syndrome was raised. She had evidence of hypogammaglobulinaemia, with an IgG level of 4.83 g/L, IgA of 0.27 g/L and IgM of < 0.2g/L, consistent with Good’s syndrome.
Results
She continued to require red cell transfusions to maintain an adequate haemoglobin level. She eventually agreed for a thymectomy 9 months after the initial diagnosis. She received red cell transfusions and intravenous immunoglobulin prior to surgery. The histology of the anterior mediastinal mass was consistent with a WHO type AB thymoma. She was commenced on immunosuppressive therapy with cyclosporine as she remained transfusion dependent despite the thymectomy. After 8 months of immunosuppressive treatment post thymectomy, she is currently transfusion dependent for 9 months.
Conclusion
Good's syndrome which was associated with the thymoma was important to exclude in this patient prior to her thymectomy to reduce the risk of post surgical infective complications. Given that infective complications were the major cause of death in a case series, I believe that thymoma-associated PRCA should be screened for Good's syndrome prior to their surgery to improve patients clinical outcome.
Session topic: 12. Bone marrow failure syndromes incl. PNH - Clinical
Keyword(s): Anemia, Immunodeficiency, Pure red cell aplasia, Thymus
Abstract: PB1841
Type: Publication Only
Background
Acquired pure red cell aplasia (PRCA) is a rare condition and is known to be associated with thymomas. Other associations have been described such as drugs, autoimmune conditions, malignancies and ABO incompatible stem cell transplants.
Aims
Here, we describe an unusual case of PRCA which was associated with both a thymoma and parvovirus infection. As part of the work-up for her thymoma, she was also found to have hypogammaglobulinaemia (Good's syndrome) and positive anti-acetylcholine receptor antibodies without clinical evidence of myasthenia gravis.
Methods
A 45-year-old lady presented with symptomatic anaemia to the haematology clinic. She had no past medical history of note prior to this consultation. She presented with symptoms of a viral illness at another hospital a few weeks prior and was found to have anaemia requiring transfusion.
On examination, she was well with mild pallor. The rest of her physical examination was unremarkable with no lymphadenopathy or hepatosplenomegaly. Her heart rate was 87 beats per minute (bpm) , blood pressure was 100/ 53 mmHg and her oxygen saturation was 100% on room air. Initial investigations revealed a normochromic, normocytic anaemia with a haemoglobin level of 8.3g/dL. Her white blood cell count (WBC) was 4.23 X 109/L and absolute neutrophil count (ANC) of 2.25 X 109/L. Her platelet count was normal. There was also reticulocytopenia with an absolute reticulocyte count of 5.80 X 109/L. The peripheral blood film showed a normochromic, normocytic anaemia with no other abnormalities seen.
A bone marrow examination was performed and revealed an absence of erythropoiesis with preserved granulopoetic and megakaryopoietic activity. Parvovirus PCR was positive as well from the bone marrow sample. A chest computed tomography (CT) demonstrated an anterior mediastinal mass (Figure 1). Given the CT findings, conditions associated with a thymoma was screened for and she was found to have a positive anti-acetylcholine receptor antibody titre of 1.76 nmol/L . Flow cytometry of the bone marrow showed B cell aplasia and the possibility of Good’s Syndrome was raised. She had evidence of hypogammaglobulinaemia, with an IgG level of 4.83 g/L, IgA of 0.27 g/L and IgM of < 0.2g/L, consistent with Good’s syndrome.
Results
She continued to require red cell transfusions to maintain an adequate haemoglobin level. She eventually agreed for a thymectomy 9 months after the initial diagnosis. She received red cell transfusions and intravenous immunoglobulin prior to surgery. The histology of the anterior mediastinal mass was consistent with a WHO type AB thymoma. She was commenced on immunosuppressive therapy with cyclosporine as she remained transfusion dependent despite the thymectomy. After 8 months of immunosuppressive treatment post thymectomy, she is currently transfusion dependent for 9 months.
Conclusion
Good's syndrome which was associated with the thymoma was important to exclude in this patient prior to her thymectomy to reduce the risk of post surgical infective complications. Given that infective complications were the major cause of death in a case series, I believe that thymoma-associated PRCA should be screened for Good's syndrome prior to their surgery to improve patients clinical outcome.
Session topic: 12. Bone marrow failure syndromes incl. PNH - Clinical
Keyword(s): Anemia, Immunodeficiency, Pure red cell aplasia, Thymus