Perioperative normovolemic hemodilution in a case of leiomyomatous erythrocytosis.

A 66-year-old female patient presented with complaints of abdominal discomfort, pigmentation and numbness of both lower limbs for 3 years duration. On examination, she had erythema of the face and palms. Investigations revealed high hemoglobin (Hb), hematocrit (Hct) and erythropoietin. Ultrasonography abdomen showed large uterine fibroid. As there are increased tendencies of thromboembolic episodes in patients undergoing surgeries with such high Hb and Hct, a target to achieve a Hb of 15g/dl and Hct of 45 was set in the patient. Repeated phlebotomies were done over 10 days with oral hydration only and the Hb was brought down to 18 g/dl on the day prior to surgery. On the day of surgery, pre-operative phlebotomy was done so as to remove 2 units of 350 ml blood and was transfused intraoperatively to combat blood loss. Post-operatively Hb was 12.4 g %. Patient was discharged on the 10(th) post-operative day with further follow-up evaluations being uneventful.

Case Report

A 66-year-old female, mother of two children was admitted to our hospital with a progressive swelling of the abdomen over 3 years, numbness and darkness of both lower limbs for 3 weeks. She had attained menopause 15 years back and there was no history of any post-menopausal bleeding. There was no history of headache, giddiness, or blurring of vision. There was no history of easy bruising or bleeding. There was no history of loss in appetite, general malaise, fever, persistent cough, weight loss, any breathing difficulty, chest pain. There were no neurological or cognitive deficiencies. There was no history of pruritus post-bathing, history of residing in high altitude or history of smoking or taking pan or any addictive substances. She was diagnosed of having hypertension 2 years back and was on treatment for the same. There was no history of diabetes mellitus, bronchial asthma, hypercholesterolemia, coronary artery disease. There was no history of any malignancies or tuberculosis in the past. There was no history of long term intake of any drugs. There was neither history of any malignancies in the family nor any hematological disorders in the family. There was no other relevant past history. She gave history of intake of mixed diet.

On examination, she seemed in good health, was moderately built and moderately nourished. She had erythema of the conjunctiva, no icterus, cyanosis, clubbing and edema or generalized lymphadenopathy. Her weight was 59 kg. Her blood volume was estimated to be around 4500 ml. Her blood pressure was 140/90 mm Hg. Her pulse was 84/min and regular. She was afebrile and had a respiratory rate of 12/min. Systemic examination of the heart, lungs and central nervous system revealed nothing abnormal. Abdominal examination revealed an irregular mass arising out of the pelvis and reaching to the level just above the umbilicus. It was firm, dull to percussion and non-tender, with restricted mobility. There was no evidence of ascites. The liver and spleen could not be palpated.

On pelvic examination, the pelvis was found to be occupied by the mass and it was about 28 weeks in size. The uterus could not be felt apart from the mass, but, by displacing the whole mass upwards with the abdominal hand, the cervix was felt to move with it and the mass was, therefore, considered to be part of the uterus. The adnexa could not be palpated. Rectal examination confirmed that the pouch of Douglas was completely occupied by the mass.

Investigations

The hemoglobin level was 22 g/dl. The hematocrit was 66 %; the white cell count was 5200/cu mm. (Differential count: Neutrophils 55%, eosinophils 8%, lymphocytes 30%, monocytes 1%); and platelet count was 1,86,000/cu mm. The blood urea concentration was 23 mg/dl. The electrocardiogram was normal and X-ray examination of the chest and abdomen was normal. Serum erythropoietin was done and was found to be raised to 40.10 mu/ml. Ultrasonography of the abdomen showed-a large solid abdomino pelvic mass containing cystic spaces and minimum internal vascularity-likely fibroid mass. Uterus and ovaries were not seen separately.

Diagnosis

A provisional diagnosis of large uterine fibromyomata with secondary polycythemia and systemic hypertension was made.

Management

A multi-disciplinary approach was planned. As her hemoglobin (Hb) and hematocrit (Hct) was high; and due to increased tendencies of thromboembolic episodes on doing surgery in such patients it was decided to bring down the Hb and Hct to a target level of 15 g/dl and 45% respectively. Repeated phlebotomies were done on every other day over 10 days and Hb was brought down to 18 g/dl on the day of surgery. She was advised to hydrate herself by drinking plenty of fluids on all these days. On the operation table, just before laparotomy, phlebotomy was done so as to remove 2 units of 350 ml blood. She was replenished by normal saline infusion so as to combat any hypovolemia. It was planned to do a total abdominal hysterectomy. Laparotomy was performed; the abdomen was opened by a vertical midline incision extending to five inches above the umbilicus. A very large fibroid uterus was delivered into the wound [figure 1]. Total hysterectomy and bilateral salpingooophorectomy was performed. Palpation of the other abdominal organs showed no abnormal pathology. Two units of blood were reinfused back to combat the loss due to bleeding during surgery on removal of the mass. The time from phlebotomy for blood collection to start of reinfusion was 2 hours, where in the blood was stored in the refrigerator in the operation theatre.

Figure 1

Large fibroid being removed by surgery

Pathology report

The specimen weighed 6.5 kg. The ovaries and tubes were normal but the cervix showed evidence of chronic cervicitis. The uterus showed gross fibromyomata with no evidence of sarcomatous degeneration

Post-operative progress

Patient's recovery was uneventful. On the 2nd day, the Hb had gone down to 12.4 g/dl. She was discharged on the 10th post-operative day. She was instructed to return at monthly interval until further notice, for routine hemoglobin and hematocrit readings. Patient came for review to the Out-patients Department after 6 weeks and her Hb was found to be 10.9 g/dl

Discussion

The term polycythemia is used to describe an increase in the number of red cells per unit volume of blood accompanied by an increase in the Hb and packed-cell volume values of the blood.[1] In polycythemia vera (primary polycythemia), the fundamental abnormality is hyperplasia of the precursor of the red cells.[2] Secondary polycythemia or erythrocytosis is a true polycythemia resulting from some known underlying primary disorder. Most of the cases are due to disorders which lowers the arterial oxygen saturation of the blood and a few are due to disorders which appears to cause erythropoietic factor production with normal oxygen saturation.

Conclusion

The association of polycythemia with uterine fibroids is rare, even though fibroids are quite a common condition. It's mainly because many practitioners are not aware of this association.[3] If a careful watch would be made on the pre-operative blood findings in the future, many more cases might be picked up.

Autologous transfusion is an untapped economical practice which can be utilized safely and effectively for various elective surgical procedures.

In this case, phlebotomy is a mode of therapy in itself thereby transfusion medicine center has a dual role to play.

The primary impetus to avoid allogeneic transfusion is no longer limited to safety alone, but of immense value in individualized patients as evident here.