The follicular lymphoma is a monoclonal tumor from the mature B-cells occurring from the follicular center of lymph nodes. A follicular lymphoma - a most often meeting variant among lymphoma.
According to the literature, loss of a part of a X-chromosome, and also the genes located on it which are responsible for formation of immunity and a hormonal background, can lead to occurrence of hemoblastoses.
The true hermaphroditism (syndrome of bisexual gonads) among other forms of anomalies of sexual development meets seldom enough. Characteristic basic line of this pathology is presence at an individual simultaneously both man´s, and female elements of a gonad. The pathology can be suspected at the child already at a birth owing to an uncertain structure of external genitals.
However the histological conclusion is the basic criterion for the definitive diagnosis.
For an illustration of the told it is resulted following observation.
Patient К., 21 year (a genetic card № 28 569).
Was born from II births in time at the young parents consisting in not related marriage. Mass at a birth 3000,0 g, the length of a body - 52 centimeters. According to mum, the proband floor at a birth raised the doubts, but has been defined as female, and the child was brought up as the girl. Proband development didn´t differ from age criteria. Sexual development proceeded on female type. A menarche since 15 years, regular, very plentiful.
From 20 years the proband is observed by a hematologist with the diagnosis the Follicular lymphoma. Notes insignificant augmentation of peripheric inguinal lymphonoduses which tend to decrease and again to arise. The patient of specific treatment didn´t receive, the doctor had been chose tactics of active observation.
At the age of 21 years of the patient concerning a purulent peritonitis the laparotomy has been spent. The purulent tumor of an ovary on the right is found out. The Suppurative focus has been removed. Histological research of a sexual gland is conducted.
Result of histological research № 40849-53 from 19.10.2000. The fine fragment of cortical substance of an ovary with an individual cavity is defined. The cavity has one layer of flat follicular cells. And also there is a clump luteocytic - a fragment of a menstrual yellow body. Cellular elements of atypical character in a remote material it is not taped.
On consultation at the doctor of the geneticist. At survey at the age of 21 years: growth of 153 centimeters, mass of 54 kg. A proband phenotype, psychological orientation, a voice timbre female. Embryogenesis deviations: low growth of hair on a forehead, a thin curly hair on a head, a small mouth, a micrognatia, dissymmetric muscles sternokleidomastoideus.
Condition satisfactory, position active. Signs of an intoxication, loss of mass of a body, a fervescence it is not traced. Integuments pure, moderately wet. The thyroid gland is moderately diffusively enlarged, painless. At a palpation a stomach soft, painless on all extent. The liver, a lien aren´t enlarged. At the left the painless individual inguinal lymphonodus, the size 10´10 mm, an elastic consistence is palpated.
Secondary sexual signs are developed on female type. Mammary glands are developed enough, the secretory tissue is palpated. At pressing from papillas separated isn´t present. The sexual formula of proband Ax3 Pb3 Ma3 Me+15. Hirsutism number- 8 points.
In the gynecologic review wrong development of external genitals on uncertain types is taped. A line of horizontal hair, on female type. Pilosis on a pubis the sufficient. The big and small sexual labiums aren´t differentiated and represent dermal cords in some kind of not completely made scrotum. The clitoris looks like not enough developed bent penis, to 6 sm in length, with the truncated chorda. At head opening in «the penis» center the punctual aperture of the urethral channel is defined. From the channel the urine drop is allocated. Back from «scrotum» the median perineal seam is located. It comes to an end with an external vaginal opening. At vagina sounding the bellied probe passes to 1 centimeter. External aperture of a female urethra see isn´t visualized. At effort from a vagina urine is allocated. The proband says that the menstrual blood also is allocated from the given aperture. Between an external vaginal opening and a fundament there is a thin plaited septum. At the penis basis dense elastic formation of the roundish form in the sizes 15×15 mm, it not soldered to surrounding tissues is palpated. At rectal research are palpated 2 dense painless uteri of the usual sizes ~ 45-50 mm.
Ultrasonic research of a uterus and its appendages. (Idea АU4). 8.02.2001.
2 bodies of the womb are accurately located: the right uterus in the size of 54-37-32 mm, endometrius linear 8 mm; the left uterus 58×39×30, endometrius linear 8 mm. 2 necks of a uterus, 2 cervical channels are accurately located. At the left the ovary in the size of 53×33×30 mm with set of liquid includings in the size to 10-15 mm is located. The conclusion: Full doubling of a uterus.
Ultrasonic research of adrenals. Adrenals of the usual sizes and structure.
Ultrasonic research of external genitals. In the basis of a root of a penis roundish formation with accurate contours with homogeneous internal structure in the size of 15×15 mm is located. The blood flow in formation isn´t defined. The conclusion: the rudimentary testicle isn´t excluded.
Research of hormones of a blood from 3/12/2001: ТТГ - 1,36 mme/ml; Testosteron-Depotum - 0,93 nmol/l; Oestradiolum - 48,0 nmol/l; Prolactinum - 231 mme/l.
The developed analysis of a blood (12.03.2001) within normal indicators. Pathological deviations it is not revealed.
Karyotype of a proband №54 from 2/9/2001 - mos 46, XX [34]; 46, X, del Xq21 [4].
The diagnosis has been made: «The Syndrome of bisexual gonads, a variant of presence of gonads of a different floor (a true hermaphroditism). True doubling of a uterus and vagina. Suspicion on an urogenital sine. An anomaly syndrome in half-defining chromosomes with structural reorganization. A chronic follicular lymphoma, an initial stage».
The patient has been referred to an urology department for specification of the diagnosis and operative excision of formation with the subsequent histological research of a material. Presence of tumoral formation of lymphomatous character isn´t excluded.
Thus, in the given observable case though androgenic activity of a testicular component of gonads is less expressed, than activity of estrogens, nevertheless, masculinization elements at a true hermaphroditism can be traced also. It is necessary to remember that at any forms of an embryonal pathology of development of gonads, tumoral changes in them are frequent enough. The situation can be aggravated with a progressing lymphoma.
The work was submitted to the International Scientific Conference «Fundamental research», Croatia, 25 July ‒ 1 August, 2011, came to the editorial office 30.05.2011.