Autoimmune hemolytic anemia and ovarian dermoid cysts in pregnancy.
Case Report. A 24-year-old, primigravida was referred to us at 24 weeks of gestation, as a case of AIHA, first diagnosed during pregnancy. Initially she was presented with fatigue and dizziness and low hemoglobin (Hgb) with no history of any medical illness or surgery. She had not been started on any new medications and had no family history of hematological diseases. Initial laboratory tests showed white blood cell (WBC) count 22.9 x [10.sup.9]/L, red blood cell (RBC) count 2.68 mcL, hemoglobin (Hgb) 7.7 g/dL (normal: 120-160 gm /L, platelet count 545000 (normal: 150-400X10 [conjunction] 9 /L), reticulocyte percentage 18.63 (normal: 0.5-2.5%), lactate dehydrogenase (LDH) 567 U/L (normal: 125-243U/L), and total bilirubin 44.1 (normal: 3.4-20.5 umol/L. Kidney function tests were within normal limits, and the direct Coomb's test was positive. Viral serology, anti-nuclear antibodies, anti-double stranded DNA antibodies, anti-lupus, anti SS-A, anti SS-B were all negative. Ultrasound showed a normal fetus with size corresponding to gestational age. However, the maternal right ovary was enlarged, measuring 7.7 x 7 cm. A complex cyst measuring 6.7 x 6 cm was seen within the ovary, and was thought to be a dermoid cyst (Figure 1).
She received high dose steroids (started by prednisolone 100 mg) with no significant improvement. Rituximab once a week four doses and intravenous immunoglobulin 3 doses were added, without any success. Hemolysis was refractory a trail of Azathioprine post delivery. Transfusion of multiple packed red blood cells (PRBCs) reached 44 units.
During follow up, the patient received a total of 44 units of PRBC for refractory low Hgb level. At 36-37 weeks of gestation, ultrasound revealed fetal pericardial effusion, and a slightly enlarged dermoid cyst.
Induction of labor was carried out and she uneventfully delivered a healthy baby vaginally. Postnatal echocardiography revealed no pericardial effusion and no other significant abnormalities.
Postpartum, Hgb level dropped from 11.0 g/dl to 7.0 g/dl. Azathioprine and prednisone, along with 6 units of PRBC were transfused. Two weeks later, the patient was seen on outpatient basis, with condition having worsened, with Hgb level having dropped further, to 6.1 g/dl.
Laparoscopic right ovarian cystectomy was performed and intraoperative findings of right ovarian dermoid cyst were confirmed on histopathological examination. Postoperatively, she improved significantly; signs and symptoms of anemia reduced and Hgb level increased up to 13.8 g/dl, while LDH levels dropped to 169 (Table 1).
Discussion. Association between dermoid ovarian cyst and AIHA is still a rare phenomenon (7) with a limited number of cases reported in the literature. Ovarian teratomas are relatively common, but the incidence of associated hemolytic anemia is low.
This phenomena was reported by West-Watson and Young (8) but reported cases are still limited especially in pregnancy. There are different hypothesis describing the immunological reaction triggered by the tumor. Several reports support the hypothesis that the tumor produces antibodies against red blood cells. Antibody production seems to cease immediately after tumor removal in almost all reported cases. (7)
Glucocorticoids and splenectomy known as the mainstay of treatment of AIHA. In our case; however, it proved to be ineffective. An improvement was seen only after tumor removal. (3-6) Payne et al (9) in 1981 have carried out a literature review of cases reported as dermoid cysts with AIHA, and collected around 19 cases. Sixteen patients responded favorably to tumor removal alone. Negligible response to steroids was reported in 3 patients who required dermoid removal to achieve full response. Antibodies were reported to disappear in 2 weeks to 7 months after tumor removal, which may be considered as a confirmation of the association. (9) Our patient was pregnant, and literature review did not reveal any case of AIHA due to a dermoid cyst, diagnosed in pregnancy. In fact, cases of AIHA during pregnancy, caused by factors other than dermoid cysts, have been reported late in pregnancy, and lay emphasis on good response to corticosteroids and blood transfusion. (5) Autoimmune hemolysis in pregnancy complicates 1 in 50000 pregnancies. (10) In most cases uneventful maternal recovery with good fetal outcome has been reported, except in a few cases caused by autoimmune diseases like systemic lupus erythematosus. (10) Owing to a lack of similar cases in literature, maternal and fetal course was closely followed, with clinical monitoring of maternal condition by symptoms and laboratory investigations, and keeping a watch for possible fetal anemia. An obstetric ultrasound showed fetal pericardial effusion at 37 weeks of gestation. However, the fetus was born in a good condition, with normal echocardiography 2 weeks after birth. Unfortunately, the maternal condition did not improve and she required repeated blood transfusions post delivery. Laparoscopy dermoid cystectomy was carried out, after which, complete recovery of the mother took place (Figure 2).
In conclusion, AIHA caused by a dermoid cyst is a rare condition. However, in light of similar case reports and review of the existing literature, it would be reasonable to conclude that in the presence of AIHA and ovarian teratoma, surgical excision should be considered. However, it should be kept in mind that this association may occur during pregnancy. Furthermore longer follow, up duration is needed to actually prove that the ovarian tumor resection led to persistent resolution of AIHA.
Acknowledgment. We would like to acknowledge Dr. Hytham Alsum, Consultant, Maternal Fetal Medicine, National Guard Health Affairs, Riyadh, Kingdom of Saudi Arabia and the Editage.com for the English editing.
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(2.) Sallah S, Sigounas G, Vos P, Wan JY, Nguyen NP. Autoimmune hemolytic anemia in patients with non-Hodgkin's lymphoma: characteristics and significance. Ann Oncol 2000; 11: 1571-1577.
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(4.) Kim I, Lee JY, Kwon JH, Jung JY, Song HH, Park YI, et al. A case of autoimmune hemolytic anemia associated with an ovarian teratoma. J Korean Med Sci 2006; 21: 365-367.
(5.) Lauzikiene D, Ramasauskaite D, Luza T, Lenkutiene R. Pregnancy induced autoimmune warm antibodies hemolytic anemia: A case report. Geburtshilfe Frauenheilkd 2015; 75: 1167-1171.
(6.) Agarwal V, Sachdev A, Singh R, Lehl S, Basu S. Autoimmune hemolytic anemia associated with benign ovarian cyst: a case report and review of literature. Indian J Med Sci 2003; 57: 504-506.
(7.) Raimundo PO, Coelho S, Cabeleira A, Dias L, Goncalves M, Almeida J. Warm antibody autoimmune hemolytic anemia associated with ovarian teratoma. BMJ Case Rep 2010; 2010: bcr06.2009.1971.
(8.) West-Watson WN, Young CJ. Young CJ. Failed splenectomy in acholuric jaundice and the relation of toxaemia to the hemolytic crisis. BMJ 1938; 1 (4041): 1305-1309.
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Afaf A. Felemban, MD, Zuha A. Rashidi, MD, Musab H. Almatrafi, MD, Jawaher A. Alsahabi, MD.
From the Reproductive and In Vitro Fertilization Unit (Felemban), and from the Obstetrics and Gynecology (Rashidi, Almatrafi, Alsahabi) National Guard Health Affairs, Riyadh, Kingdom of Saudi Arabia.
Received 8th September 2018. Accepted 20th March 2019.
Address correspondence and reprint request to: Dr. Afaf A. Felemban, Reproductive and In Vitro Fertilization Unit, National Guard Health Affairs, Riyadh, Kingdom of Saudi Arabia. E-mail: Afelemban@hotmail.com ORCID ID: https:llorcid.orgl0000-0002-2149-U64
Disclosure. Authors have no conflict of interests, and the work was not supported or funded by any drug company.
Caption: Figure 1--Right sided ovarian dermoid cyst.
Caption: Figure 2--Laboratory results. Hb--hemoglobin (g/dl). LDH--lactate dehydrogenase (U/L), Bili T--total bilirubin, Retic--reticulocyte percentage
Table 1--Timeline of summary of the case. Date/status Symptoms/signs Investigations 4th January Initial visit and Hemoglobin 7.7 (after received 24 weeks referral: 25 years 2 units of packed red blood pregnant old prim gravida 24 cells in her local hospital) weeks diagnosed and referred as AIHA Complaining of sever weakness and fatigue On examination pale and jaundiced 11 January Sever fatigue and Hemoglobin = 67 weakness Hematocrit = 0.207 Lactate dehydrogenase = 571 Total bilirubin = 44 Absolute reticulocyte count = 574.4 19 January -5th Not documented Hemoglobin = 79 February Hematocrit = 0.222 Lactate dehydrogenase = 638 Total bilirubin = 36 Absolute reticulocyte count = 492.8 1st March Not documented Hemoglobin = 76 32 weeks Hematocrit = 0.218 Lactate dehydrogenase = 646 Total bilirubin = 33.7 Absolute reticulocyte count = 604.8 5 th April Induction of labor Hemoglobin = 86 37 weeks and Vaginal delivery Hematocrit = 0.24 pregnant Lactate dehydrogenase = 975 Total bilirubin = 33.9 Absolute reticulocyte count = 517.6 20 April Not documented Hemoglobin = 73 2 weeks post Hematocrit = 0.222 partum Lactate dehydrogenase = 921 Total bilirubin = 41 Absolute reticulocyte count = 791.7 8th May Hemoglobin = 66 5 weeks post Complaint of Hematocrit = 0.2 partum dizziness Lactate dehydrogenase = 911 Total bilirubin = 50.6 Absolute reticulocyte count = 10th May 5 647 weeks post partum 24 may Feeling better no Hemoglobin = 119 2 weeks post complaints Hematocrit = 0.355 operative Jaundice and pallor Lactate dehydrogenase = 293 improved Total bilirubin = 17.1 Absolute reticulocyte count = 95.8 13 June Feeling better no Hemoglobin = 139 5 weeks post complaints Hematocrit = 0.421 operative Lactate dehydrogenase = 230 Total bilirubin = 12.5 Absolute reticulocyte count = 58.3 Date/status Blood transfusion Intervention 4th January None 100 mg Prednisone for 3 24 weeks days 70 mg Prednisone pregnant (4/1-20/1) 11 January 11- 17 January Intravenous 10 units PRBCs (1-3 immunoglobulin 2 doses of unit per day) each 700000 mg 19 January -5th 22 January--28 4 doses Rituximab 700 mg February February 15 units of 50 mg Prednisone (20/1-26/1) packed red blood cell 40 mg Prednisone (27/1--1/2) 30 mg Prednisone (3/2--6/4) 1st March 1st March -26 March One dose intravenous 32 weeks 7 units of packed red immunoglobulin 20 mg blood cell Prednisone 5 th April 2 April received 2 Prednisone tapered down to 5 37 weeks units of packed red mg then discontinued pregnant blood cell 20 April 20th April received 2 Azathioprine for 30 days 2 weeks post units packed red partum blood cell 1st May received 3 units packed red blood cell 8th May 9th May received 2 units 5 weeks post packed red blood cell partum 10th May received 1 unit packed red blood cell 10th May 5 Laparoscopic ovarian weeks post cystectomy partum 24 may No transfusion 2 weeks post required operative 13 June No transfusion 5 weeks post required operative
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|Title Annotation:||Case Report|
|Author:||Felemban, Afaf A.; Rashidi, Zuha A.; Almatrafi, Musab H.; Alsahabi, Jawaher A.|
|Publication:||Saudi Medical Journal|
|Date:||Apr 1, 2019|
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