|Year : 2013 | Volume
| Issue : 3 | Page : 162-163
Anesthetic management of a pregnant patient with a pure red cell aplasia
Channabasavaraj S Sanikop, Prerna Bansal
Department of Anesthesia, J.N. Medical College, KLE University, Belgaum, Karnataka, India
|Date of Web Publication||19-Oct-2013|
Department of Anesthesia, J.N. Medical College, KLE University, Belgaum, Karnataka
Source of Support: None, Conflict of Interest: None
Pure red cell disorder is an uncommon disorder in which maturation arrest occurs in the maturation of erythrocytes. Erythroblats are virtually absent in the bone marrow. Surgery poses a very high-risk for these patients because of the several complications that can occur in the perioperative period. In this case report, we report a pregnant patient with a pure red cell aplasia who was optimized pre-operatively and underwent cesarean section under sub-arachnoid block.
Keywords: Pure red cell aplasia, sub-arachnoid block, pregnancy
|How to cite this article:|
Sanikop CS, Bansal P. Anesthetic management of a pregnant patient with a pure red cell aplasia. J Sci Soc 2013;40:162-3
| Introduction|| |
Pure red cell aplasia is characterized by a selective decrease in erythroid pre-cursor cells in the marrow and by peripheral blood anemia. The decrease is thought to occur due to selective hypoproliferation of the committed erythroid progenitor cell. Pure red cell aplasia can be acquired or inherited. It is diagnosed by profound chronic normochromic, normocytic or macrocytic anemia with decreased or absent reticulocytes. Cellular marrow with markedly decreased to absent nucleated red blood cells (RBC), but normal granulocytic and megakaryocytic cells. There is a normal peripheral total leucocyte count and differential count, normal number of platelets and absence of hemorrhagic phenomena and evidence of extra medullary hematopoiesis.
| Case Report|| |
A 32-year-old female a known case of pure red cell aplasia since 2 years presented with full term pregnancy for safe institutional delivery. She was admitted in a tertiary care hospital for anemia 2 years back.  She underwent a series of investigations to evaluate the cause of anemia. The investigations were carried out to rule out thalassemia, glucose-6-phosphate dehydrogenase (G6PD) deficiency, sickle cell anemia, and multiple myeloma. Coomb's test, acid ham test, sucrose lysis test, paroxysmal nocturnal hemoglobinuria (PNH), anti-MIRL (CD59), anti-DAF (CD55) tests, and urine bence jones proteins were performed and all reports were negative. The diagnosis of pure red cell aplasia was made on these findings.
Bone marrow examination carried out one year back and the report was as follows, Cellularity Good, M:F ratio-35:1, myeloid series: Normal, orderly maturation, no blast, no dysplasia, erythroid series: Severely depleted, occasional pre-normoblasts, megakaryocytes: Adequate, plasma cells: 1%, lymphocytes: 14%, iron stain: +++, serum iron -121 μg/dl (40-140 μg/dl), total iron binding capacity (TIBC) -171 μg/dl (245-400 μg/dl), transferrin saturation -70.8% (16-50%), serum ferritin -1025 ng/dl (males 28-397 ng/dl and females 6-159 ng/dl), intrinsic factor blocking antibody -3 (negative <12, equivocal 12-18, positive >18), anti-nuclear antiibody and anti-dsDNA antibody was negative.
Serum erythropoietin - >2000 mIU/ml (8.9-29.5 mIU/ml), vitamin B 12 -1039 pg/ml (200-950 pg/ml), red cell folate -720ng/ml (175-700 ng/ml), at the time of admission in Bombay hospital her hemoglobin was 3.2 g/dl. She was transfused with 12 points of blood after diagnosing pure red cell aplasia.  The patient was on steroids and was not having any symptoms of disease since then. One year later she conceived. She came to hospital with complains of amenorrhea. Her pregnancy was diagnosed and confirmed by ultrasonographic studies and last menstrual period (LMP) being 29 January 2012. Pregnancy was uneventful and she had regular ante-natal check-ups. Her blood picture was regularly monitored during the course of pregnancy. Expected date of delivery was 5 November 2012. The patient presented to hospital for safe institutional delivery. At the time of cesarean section her hemoglobin was 10.8 g% hematocrit 32.7%, white blood cells (WBC) count 10,700 cells/mm 3 , platelet count 1.2 lakhs/mm 3 , erythrocyte sedimentation rate (ESR) 22 mm at the end of 1 h. Reticulocyte count was 0.8%, RBC 3.74 million/mm 3 , mean corpuscular volume (MCV) 87.2 fl, MCH 28.8 pg, mean corpuscular hemoglobin concentration (MCHC) 33%, red blood cell distribution width (RDW) 12.6%. There was no hypochromia, microcytosis, macrocytosis, anisocytosis, and other morphological changes of RBC. Differential WBC count was normal. Her routine urine investigations, mini renal profile, liver function tests, and electrocardiography (ECG) were within normal limits. Her pulse rate was 74/min, blood pressure 110/70 mmHg, respiratory rate was 16/min, chest was clear and heart sounds were normal. She was on tablet methyl prednisolone 5 mg (alternate day), tab folvite 5 mg, tab methylcobalamine, tab rocaltrol (calcitriol), and tab livogen.
We decided to manage the case by giving sub-arachnoid block. Sub-arachnoid block was given under strict asceptic pre-caution using 23 gauge quincke spinal needle in L3-L4 sub-arachnoid space and 1.8 cc of 0.5%(H) bupivacaine was injected after confirmation of free flow of cerebrospinal fluid (CSF). Monitoring included a continuous electrocardiogam, non-invasive blood pressure, oxygen saturation, respiratory rate, and urine output. The surgery was uneventful and lasted for 60 min. Surgical blood loss was around 800 ml. Patient remained stable throughout the procedure with adequate urine output. Perioperative course was uneventful and the patient was discharged on 5 th post-operative day when her hemoglobin was 9.5 g%.
| Discussion|| |
Pure red cell aplasia  is caused by a selective destruction or inhibition of erythroid progenitor or precursor cells. It is characterized by anemia and reticulocytopenia and occurs as an acute or chronic condition. As in the case of all autoimmune disorders, the results of treatment with glucocorticoids and cytotoxic drugs or immunosuppressive agents are unpredictable and treatment often leads to serious complications.
Transfusion with packed red cells is the mainstay of symptomatic therapy. A hemoglobin concentration maintained at 8-10 g/dl is an attainable goal in complete erythroid aplasia. In our patient, the hemoglobin was maintained pre-operatively at 10.8 g%.
Steroid hormones like glucocorticoids  are frequently effective in reactivating red cell production either by blocking antibody production or by sensitizing abnormal erythroid progenitor cells to normal growth factors. Unfortunately, rather substantial doses may be needed, and side-effects often preclude the continuous employment of these drugs. When small maintenance doses are effective; however, glucocorticoid treatment can eliminate transfusion dependence and be the treatment of choice. Our patient was put on low dose of methyl prednisolone 5 mg, and iron, folic acid, methyl cobalamine and calcium supplements and she did not require any blood transfusions during the course of pregnancy.
Course and prognosis
Remissions have been induced in about 25% of patients either with or without thymomas, but only half of these have been sustained without further therapy. In most cases, maintenance therapy with transfusions, erythropoietin, and adrenal steroids has been responsible for both symptomatic control of the disease.
| Acknowledgment|| |
We would like to thank the department of Gynecology and Obstetrics of JNMC Medical College, KLE University and special thanks to Dr. M. B. Bellad for allowing us to anaesthetize this case.
| References|| |
|1.||Makino Y, Nagano M, Tamura K, Kawarabayashi T. Pregnancy complicated with pure red cell aplasia: A case report. J Perinat Med 2003;31:530-4. |
|2.||Erslev AJ, Beutler E, Lichtman MA, Coller BS, Kipps TJ, editors. Pure red cell aplasia. Williams Hematology. McGraw-Hill, New York; 1995. p. 448-56. |
|3.||Sawada K, Hirokawa M, Fujishima N. Diagnosis and management of acquired pure red cell aplasia. Hematol Oncol Clin North Am 2009;23:249-59. |