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Autoimmune hemolytic anemia and ovarian dermoid cysts in pregnancy.

There may be several causes of autoimmune hemolytic anemia (AIHA). Rare causes of AIHA are tumors, including ovarian tumors, (1,2) and these can be either malignant or benign. (3,4) We present a case of AIHA which developed during pregnancy, associated with ovarian tumor. Autoimmune hemolytic anemia is IgG Mediated disease for that it is a serious condition for mother and fetus (5) The mechanism of hemolysis in pregnancy has not yet been defined. Agarwal et al in 2003 (6) discuss the pattern of such condition and reviewed their clinical course, he reviewed 20 cases of AIHA associated with dermoid cyst, 85% of them respond to cystectomy after failed corticosteroids and one under went cystectomy shortly after diagnosis who showed some response to steroid management. However, effective treatment in our case was post delivery tumor removal. Thus, it is important for obstetricians/gynecologists to know that teratoma is one of the etiologies of AIHA during 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.

doi: 10.15537/smj.2019.4.24107

Acknowledgment. We would like to acknowledge Dr. Hytham Alsum, Consultant, Maternal Fetal Medicine, National Guard Health Affairs, Riyadh, Kingdom of Saudi Arabia and the for the English editing.


(1.) Jung CK, Park JS, Lee EJ, Kim SH, Kwon HC, Kim JS, et al. Autoimmune hemolytic anemia in a patient with primary ovarian non-Hodgkin's lymphoma. J Korean Med Sci 2004; 19: 294-296.

(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.

(3.) Glorieux I, Chabbert V, Rubie H, Baunin C, Gaspard MH, Guitard J, et al. [Autoimmune hemolytic anemia associated with a mature ovarian teratoma]. Arch Pediatr 1998; 5: 41-44.

(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.

(9.) Payne D, Muss HB, Homesley HD, Jobson VW, Baird FG. Autoimmune hemolytic anemia and ovarian dermoid cysts: case report and review of the literature. Cancer 1981; 48: 721-724.

(10.) Sokol RJ, Hewitt S, Stamps BK. Erythrocyte autoantibodies, autoimmune haemolysis and pregnancy. Vox Sang 1982; 43: 169-176.

[Please note: Some non-Latin characters were omitted from this article]

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: 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 =

19 January -5th  Not documented         Hemoglobin = 79
February                                Hematocrit = 0.222
                                        Lactate dehydrogenase = 638
                                        Total bilirubin = 36
                                        Absolute reticulocyte count =

1st March        Not documented         Hemoglobin = 76
32 weeks                                Hematocrit = 0.218
                                        Lactate dehydrogenase = 646
                                        Total bilirubin = 33.7
                                        Absolute reticulocyte count =

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 =

20 April         Not documented         Hemoglobin = 73
2 weeks post                            Hematocrit = 0.222
partum                                  Lactate dehydrogenase = 921
                                        Total bilirubin = 41
                                        Absolute reticulocyte count =

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

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 =

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 =

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

5 weeks post     packed red blood cell
partum           10th May received 1
                 unit packed red blood
10th May 5                               Laparoscopic ovarian
weeks post                               cystectomy

24 may           No transfusion
2 weeks post     required

13 June          No transfusion
5 weeks post     required
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Article Details
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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
Article Type:Report
Geographic Code:7SAUD
Date:Apr 1, 2019
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