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MODERN DISEASES AND SAFETY ISSUES IN THE TREATMENT OF
ONCOLOGICAL APLASIAS
Fayzullaeva Madina Bakhshullo kizi
Tashkent State Medical University
1
, Uzbekistan
Mamarajabova Soliha Ulugʻbek kizi
Tashkent State Medical University
1
, Uzbekistan
Sodiqova Nigoraxon Sodirjon kizi
Tashkent State Medical University
1
, Uzbekistan
Musayeva Zubayda Umar kizi
Kimyo International University in Tashkent, Uzbekistan
Annotatsiya
Bugungi kunda onkologik kasalliklar orasida aplaziya holatlari alohida ahamiyat
kasb etmoqda. Aplaziya — bu hujayralar yoki to‘qimalarning rivojlanmasligi yoki
yo‘qligi bilan kechadigan holat bo‘lib, u bir qancha xavfli o‘smalar bilan bevosita yoki
bilvosita bog‘liq bo‘ladi. Ayniqsa, gematologik yo‘nalishda uchraydigan aplastik
anemiya, miya hujayralari aplaziyasi yoki limfoid to‘qimalarning yetishmovchiligi
kabi shakllar zamonaviy onkologik amaliyotda ko‘plab muammolarni yuzaga
keltirmoqda. Davolash jarayonida immunosupressiv terapiya, giyohvandlikka qarshi
vositalar, transplantatsiya va genetik muvofiqlik muhim omillar bo‘lib qolmoqda.
Ushbu maqolada onkologik aplaziyalarning zamonaviy davolash usullari, ularning
samaradorligi, shuningdek, mavjud muammolar va ilmiy izlanishlarga ehtiyoj tahlil
qilinadi.
Kalit so’zlar:
Onkologik aplaziya, aplastik anemiya, immunosupressiv terapiya,
transplantatsiya, genetik moslik, dolzarb muammolar
Аннотация:
В настоящее время случаи аплазии в онкологии приобретают
особое значение. Аплазия — это состояние, при котором отсутствует развитие
или формирование клеток и тканей, часто связанное с различными
злокачественными
новообразованиями.
Особенно
актуальны
формы,
встречающиеся в гематологии, такие как апластическая анемия, аплазия клеток
костного мозга, дефицит лимфоидной ткани и другие. Современные подходы к
лечению включают иммуносупрессивную терапию, противоопухолевые
препараты, трансплантацию и генетическую совместимость. Однако данные
методы не являются универсальными. В данной статье рассматриваются
Ta'lim innovatsiyasi va integratsiyasi
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ISSN:3030-3621
современные методы терапии онкологических аплазий, их эффективность, а
также существующие проблемы и необходимость дальнейших научных
исследований.
Ключевые слова:
Онкологическая аплазия, апластическая анемия,
иммуносупрессия, трансплантация, генетическая совместимость, актуальные
проблемы.
Abstract:
Nowadays, cases of aplasia in oncology are gaining increasing
importance. Aplasia is a pathological condition characterized by the absence or
underdevelopment of cells or tissues, and it is often directly or indirectly associated
with malignant tumors. Particularly relevant are hematological forms such as aplastic
anemia, bone marrow cell aplasia, and lymphoid tissue deficiency. Modern treatment
approaches include immunosuppressive therapy, antitumor drugs, transplantation, and
genetic matching. This article discusses current therapeutic strategies for oncological
aplasias, evaluates their effectiveness, and highlights the urgent need for further
scientific research.
Keywords:
Oncological aplasia, aplastic anemia, immunosuppressive therapy,
transplantation, genetic compatibility, pressing issues.
Introduction:
Aplasia cutis congenita is a rare, congenital disorder. In its severe
phenotype, it is potentially life threatening. Its management and the timing of surgery
remain controversial because of the risks involved with both conservative and surgical
approaches. Most literature is based on case reports and very small case series because
of the rarity of the disorder. The authors present their experience treating newborns
with aplasia cutis congenita and its progressive development.
Aplasia Cutis Congenita
(ACC), congenital absence of the skin, is an uncommon anomaly. It most commonly
presents as a solitary defect of the scalp but may also involve the trunk and extremities.
The lesions are non inflammatory, well demarcated and have variable extents, ranging
birth. The cause is not clear but genetic factors, compromised vasculature to the skin,
infection, teratogens, fetus papyraceous and trauma are all implicated. Truncal aplasia
cutis congenita has been reported with biliary atresia, distal duodenal atresia, intestine
infarction and multiple hepatic hematomas. Syndromes such as Adams Oliver
syndrome, SCALP syndrome (nevus sebaceus, CNS malformations, aplasia cutis
congenita, limbal dermoid, pigmented nevus). [1]
Histological details are available in very few reports. Histological features vary
depending on the depth and duration of aplasia. Ulcers are seen at birth. After healing,
the epidermis appears flattened with proliferation of fibroblasts within a connective
tissue stroma. The total absence of the epidermal appendages remains a characteristic.
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Aplasia cutis congenita histology
The diagnosis of ACC can typically be made solely from clinical examination.
Many providers withhold performing a lesional biopsy given the patient's age
demographics and typical scalp involvement. When a biopsy is necessary to aid in the
diagnosis, proper workup, including imaging of the lesion with ultrasound (US) or
MRI, is important to ensure there are no underlying malformations that can be damaged
during the biopsy procedure. Histopathologic findings of non-healed lesions include
an absent epidermis and/or dermis with a proliferation of blood vessels. A subtype
known as membranous-type ACC will have a thin translucent membrane covering.
Lesions that have already healed with a scar will have a thin or flattened epidermis,
absent adnexal structures, and dense, dermal fibrosis noted on pathology.
A classification for ACC was proposed in 1986, which is still accepted today,
and presented below. [2]
Group 1: Scalp ACC without multiple anomalies
Group 2: Scalp ACC with limb abnormalities
Group 3: Scalp ACC with epidermal and organoid nevi
Group 4: ACC overlying congenital malformations
Group 5: ACC with associated fetus papyraceus or placental infarct
Group 6: ACC with epidermolysis bullosa
Group 7: ACC localized to extremities without blistering
Group 8: ACC due to specific teratogens
Group 9: ACC associated with malformation syndromes
ACC is a rare congenital condition with an incidence of approximately 1 to 3 out
of 10,000 births.[3] There is no significant gender or cultural predilection that has been
reported in the literature. [4] Lesions will typically be noticed at birth, although patients
may not present to be evaluated for several months as lesions are often asymptomatic.
ACC can be associated with underlying morphologic abnormalities in
approximately 37% of cases, according to Mesrati et al. including underlying bony
defects, vascular anomalies, or neurologic malformations, so it is prudent for clinicians
to evaluate the disease involvement with imaging. A midline vertex scalp lesion, hair
collar sign, and vascular stains have all been shown to be strong indicators for cranial
or central nervous system (CNS) involvement. Small, scalp lesions are less likely
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associated with underlying defects and typically heal on their own within a couple of
months; therefore, monitoring these lesions without further imaging is acceptable. For
larger, ulcerative lesions, ultrasound provides a relatively inexpensive evaluation while
not putting the child through a great deal of discomfort. If there is any concern for
underlying defects on ultrasound, further workup with MRI is warranted. MRI is more
sensitive and specific for identifying underlying lesions according to a 2017
retrospective multicenter study; however, it is more costly than ultrasound and
typically requires the child to be sedated for the duration of the procedure, making this
a poor choice for initial screening. If the lesion is purulent or surrounded by erythema, a
lab workup including complete blood count, blood cultures, and wound cultures would
be advised.
Although isolated ACC without an underlying defect can have a relatively
benign course when complications occur, the risk of mortality dramatically increases.
The estimated mortality rate ranges from 20% to 55% as a result of serious
complications. The most common life-threatening complication of ACC is sagittal
sinus bleeding, seen with lesions nearby on the scalp. Another potential complication
of ACC includes secondary infection of the lesion. Patients are at an increased risk of
cutaneous infections, given the fact that the skin’s barrier against environmental
microbes is absent or impaired. Severe infections can progress to meningitis if not
treated appropriately. Prompt management of large scalp lesions, commonly with
surgery, can help prevent these complications.
Aplasia Cutis Congenita: Clinical Types, Causes, and Management
No
.
Type of ACC
(Classification
)
Appearanc
e
Etiology
Associated
Syndromes
Treatment
Approach
1
Type I (only
scalp)
Hairless
scarred
zone
Isolated
(idiopathic
)
None
Monitoring, no
surgery needed
2
Type II (deep
skin defect)
Full-
thickness
skin loss
Teratogens
(drugs,
infections)
Trisomy 13
Possible
skin
grafting
3
Type III (scalp
and face)
Multiple
lesion
zones
Genetic –
AD/AR
patterns
Adams–
Oliver
syndrome
Multidisciplinar
y approach
4
Type IV (other
div parts)
Neck, back,
legs
Vascular
disruption
in skin
Limb-div
wall complex
Evaluate
for
additional
anomalies
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5
Type V (twin-
related)
Focal
defect area
Twin
gestation
with
circulatory
issues
Fetus
papyraceus
Esthetic
and
psychological
support
6
Type VI (with
EB)
Fragile,
blistering
skin
Autosomal
recessive
disorder
Epidermolysi
s bullosa
Wound care +
protective
dressings
7
Type
VII
(amniotic
bands)
Band-like
scar
formation
Amniotic
membrane
rupture
Craniofacial
defects
Surgical
intervention
required
Table 1.
Conclusion:
If imaging with ultrasound or MRI shows concern for an underlying
abnormality, surgical intervention is usually necessary to prevent complications such
as superior sagittal sinus hemorrhage, meningitis, thrombosis, among others. When
isolated cases without underlying defects are present, the prognosis is quite good with
simple
wound
care
and
close
monitoring
by
pediatricians
and/or
dermatologists.
Although complications are rare, an understanding of ACC is
important
for
providers
to effectively work-up cases
and
to ensure prompt
consultations are made when needed.
References:
1.
M Hadiuzzaman, MBBS Iran J Dermatol 2013; 16: 36-38
2.
2. Sybert, V. P. (2002). Aplasia Cutis Congenita. In: Pagon RA, Adam MP,
Ardinger HH, et al. (Eds.), GeneReviews®. University of Washington, Seattle.
Available from: https://www.ncbi.nlm.nih.gov/books/NBK1421/
3.
3. Drolet, B. A., & Frieden, I. J. (2005). Aplasia Cutis Congenita and Associated
Disorders: Diagnosis and Management. Pediatric Dermatology, 22(6), 547–553.
https://doi.org/10.1111/j.1525-1470.2005.00128.x
4.
4. van Allen, M. I., Curry, C., & Gallagher, L. (1987). Limb div wall complex: I.
Pathogenesis. American Journal of Medical Genetics, 28(3), 529–548.
https://doi.org/10.1002/ajmg.1320280312
5.
5. Johnson, B. L., & Maize, J. C. (2003). Dermatopathology: A Volume in the
Foundations in Diagnostic Pathology Series. Elsevier Health Sciences.
6.
6. Garty, B. Z., & Danon, Y. L. (1991). Aplasia cutis congenita: Case report and
literature
review.
Clinical
Pediatrics,
30(2),
104–106.
https://doi.org/10.1177/000992289103000205
7.
7. Bavnick, J. N., & Weaver, D. D. (1986). Autosomal Dominant Inheritance of
Aplasia Cutis Congenita. American Journal of Medical Genetics, 23(3), 687–694.
https://doi.org/10.1002/ajmg.1320230313
