Ecthyma Gangrenosum in Patient with Bone Marrow Aplasia: A Case Report and Review of the Literature

Background: Ecthyma gangrenosum (EG) is an infrequent and discernible cutaneous disease caused by Pseudomonas aeruginosa. In situations where it is associated with septicemia in debilitated patients, the prognosis is usually unfavorable. Objective: In this case, we aim to verify risk factors, clinical, bacteriological and therapeutic characteristics of ecthyma gangrenosum and we review the literature to highlight the features of this rare condition and discuss the role of early diagnosis and treatment. Case Report: We describe the clinical case of a 4-year-old male with bone marrow aplasia who was presented with characteristic skin lesions of EG and developed sepsis later. Conclusion: EG is a cutaneous disease characterized by its aggressive nature. The presence of delayed diagnosis and therapy, along with sepsis, is closely linked to a high mortality rate. Treatment is empirically founded on an aggressive initial approach.

Type VI Aplasia Cutis Congenita: About a Case Report at University Teaching Hospital of Bouaké

Introduction: Aplasia cutis congenita is a rare congenital dermatosis of which type VI represents the Bart’s syndrome. The aim of this case is to describe the epidemiological, clinical, therapeutic and prognostic characteristics of this condition in a country with limited resources, for the improvement of prognosis and professional practice. Observation: This is a eutrophic newborn, born at term by vaginal delivery, who presented at birth with a unilateral absence of skin on the anteromedial aspect of the right leg starting from the knee and extending to the medial aspect of the right foot, with a dystrophy of the nail of the right big toe without any other visible physical malformation. The evolution was marked at D3 of life by the appearance of bullae on the right hand and elbow as well as on the posterior aspect of the neck, making epidermolysis bullosa suspect. The mother was 38 years old, 8th gesture, 7th pare with history of consanguinity and collodion baby. The association of a localized congenital absence of skin on the lower limbs, epidermolysis bullosa and a nail anomaly led to the diagnosis of congenital cutaneous aplasia of type VI of Frieden’s classification or Bart’s syndrome. The evolution was satisfactory on the 7th day of life with the beginning of scarring. The management was medical. The outcome was unfavorable with the appearance of sepsis and hemorrhage leading to death. Conclusion: Although rare, the clinical diagnosis of Bart’s syndrome is simple. However, the management is complex and the prognosis is reserved. To improve this prognosis, the treatment must guarantee excellent control of the infectious and hemorrhagic risks, an adhesion and good therapeutic compliance by the parents and a rigorous monitoring.

Non-Chemotherapy Medullary Aplasia in the Pediatric Oncology Unit of the Gabriel Touré Teaching Hospital, Bamako

Objectives: The main objective was to study the epidemiological, diagnostic and therapeutic aspects of medullary aplasia (MA). Methods: This was a prospective and descriptive retro study conducted from January 1, 2008 to December 31, 2018 in the pediatric oncology unit of the pediatrics department of the Gabriel Touré teaching Hospital in Bamako. Results: We collected 29 children’s cases out of 1632 admissions during the study period, representing a frequency of 1.8% and an incidence of 2.6 cases per year. The sex ratio was 2.6. The 11 - 15 age group accounted for 45%, with an average age of 8.93 years. The majority of fathers (55.2%) and mothers (62.1%) had received no education;they were mainly farmers (62.1%) and housewives (86.2%). The average consultation time was 92.21 days. Anemia was the reason for consultation in 69% of cases. Pallor was present on admission in 96.5%;infectious syndrome accounted for 79.3%, anemic syndrome for 51.7% and hemorrhagic syndrome for 27.6%;the three syndromes were associated in 27.6%. Malaria was associated with bone marrow aplasia in 58.6%. Anemia was present in 93.1%, neutropenia in 65.5% and thrombocytopenia in 86.2%. All had received a labile blood product (LBP) transfusion, and 24 (83%) had received antibiotics. Patients were treated with corticosteroids (58.6%), androgens (20.7%) and immunosuppressants (20.7%). The death rate was 34.6%. At last count, 15 (83%) had discontinued treatment, 2 (11%) were undergoing treatment and 1 (6%) was in remission. Conclusion: Effective treatment of MA requires improved technical facilities and better economic conditions for parents.

Major Ear Aplasia and Cochleovestibular Dysplasia: Rare Congenital Malformation about a Case

Introduction: The congenital malformations of the ear are rare and often isolated, may be unilateral or bilateral, and can be associated with another syndromic malformation. Such malformations so not necessarily impact aesthetics and social relations. Case Presentation: The authors report the case of Samuel M, male born at 38 WA, who is the first child of healthy parents from the same socio-cultural area. His birth weight was 2800 g and he did not have any risk factors for deafness or concept of obstetrical trauma. He presented with congenital malformation manifesting as bilateral ear aplasia with unilateral facial paralysis. Computed tomography revealed abnormalities of the inner ear;functional explorations, such as PEA and OEA, showed findings in favor of bilateral cophosis. ASSR (Auditory Steady-State Responses) was not performed. The announcement of the serious diagnosis of deafness requires multidisciplinary care in order to plan a therapeutic program to limit the impact on the development of language, schooling, and consequently, the socio-professional future of children. Conclusion: This clinical case underlines the fact that interest of the clinical interview before possible multiple surgery does not always guarantee the satisfaction of the desire for repair in this type of patient.

Pure red cell aplasia due to parvovirus B19 infection after liver transplantation:A case report and review of the literature

Pure red cell aplasia （PRCA） due to parvovirus B19 （PVB19） infectiori after solid organ transplantation has been rarely reported and most of the cases were renal transplant recipients, Few have been described after liver transplantation. Moreover, little information on the management of this easily recurring disease is available at present. We describe the first case of a Chinese liver transplant recipient with PVB19-induced PRCA during immunosuppressive therapy. The patient suffered from progressive anemia with the lowest hemoglobin level of 21 g/L. Bone marrow biopsy showed selectively inhibited erythropoiesis with giant pronormoblasts. Detection of PVB19-DNA in serum with quantitative polymerase chain reaction （PCR） revealed a high level of viral load. After 2 courses of intravenous immunoglobulin （IVIG） therapy, bone marrow erythropoiesis recovered with his hemoglobin level increased to 123 g/L. He had a lowlevel PVB19 load for a 5-too follow-up period without recurrence of PRCA, and finally the virus was cleared. Our case indicates that clearance of PVB19 by IVIG in transplant recipients might be delayed after recovery of anemia.

Treating aplasia cutis congenita in a newborn with the combination of ionic silver dressing and moist exposed burn ointment: A case report

BACKGROUND Aplasia cutis congenita (ACC) in newborns is a condition in which congenital defects or hypoplasia is present in part of the epidermis,dermis and even subcutaneous tissue (including muscle and bones).First reported by Cordon in 1767,ACC is a rare disease with a low incidence of 1/100000 to 3/10000.Currently,there are 500 cases reported worldwide.ACC can be accompanied by other malformations.The onset mechanism of the disease remains unknown but is thought to be correlated to factors such as genetics,narrow uterus,foetal skin and amniotic membrane adhesion,use of teratogenic drugs in early pregnancy and viral infection.CASE SUMMARY In August 2018,we treated a newborn with ACC on the left lower limbs using a combination of ionic silver dressing and moist exposed burn ointment (MEBO) and achieved a satisfactory treatment outcome.The skin defects were observed on the external genitals and on areas from the left foot to 3/4 of the upper left side.Subcutaneous tissue and blood vessels were observed in the regions with skin defects.The following treatments were provided.First,the wound was rinsed with 0.9% sodium chloride solution followed by disinfection with povidone-iodine twice.And then MEBO was applied to the wound at a thickness of approximately 1 mm.After applying ionic silver dressing,the wound was covered with sterile gauze.The wound dressing was replaced every 2-3 d.At the 4-mo follow-up,the treatment outcome was satisfactory.There was minimal scar tissue formation,and limb function was not impaired.CONCLUSION The combination of ionic silver dressing and MEBO to ACC is helpful.

RETREATMENT WITH FLUDARABINE AND CYCLOSPORINE FOR ONE CASE OF REFRACTORY PURE RED CELL APLASIA

MANY cases of pure red cell aplasia （PRCA） were mediated by over-function of immune cells, and responded well to immunosuppressive therapy. Sometimes refractory cases also arose. Fludarabine is an analogue of adenosine resistant to deamination which is widely used for B-chronic lymphocytic leukemia （CLL） and other hematological malignancies.^2 As a strong immunosuppressive agent, fludarabine has generally been used in nonmyeloblative conditioning regimens for hematopoietic stem cells transplantation for hematological malignancies and severe aplastic anemia recently.^3 In this study,

Pure red cell aplasia caused by pegylated interferon-α-2a plus ribavirin in the treatment of chronic hepatitis C

Pure red cell aplasia (PRCA) is a rare hematological disorder which is characterized by severe anemia,reticulocytopenia and almost complete absence of erythroid precursors in bone marrow.The pathophysiology of PRCA may be congenital or acquired.To our knowledge,there is only one case report in the English literature of PRCA after pegylated interferon combination therapy for chronic hepatitis C.We report a second case of PRCA after pegylated interferon combination treatment for chronic hepatitis C.The diagnosis of PRCA was confirmed by the typical findings of bone marrow biopsy.The possible etiologies of our case are also discussed in this paper.

Hepatitis E virus-related acute liver failure associated with pure red cell aplasia

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Angioimmunoblastic T-cell lymphoma-associated pure red cell aplasia with abdominal pain

Angioimmunoblastic T-cell lymphoma(AITL)is a unique type of peripheral T-cell lymphoma with a constellation of clinical symptoms and signs,including weight loss,fever,chills,anemia,skin rash,hepatosplenomegaly,lymphadenopathy,thrombocytopenia and polyclonal hypergammaglobulinemia.The histological features of AITL are also distinctive.Pure red cell aplasia is a bone marrow failure characterized by progressive normocytic anemia and reticulocytopenia without leucopenia or thrombocytopenia.However,AITL with abdominal pain and pure red cell aplasia has rarely been reported.Here,we report a rare case of AITL-associated pure red cell aplasia with abdominal pain.The diagnosis was verified by a biopsy of the enlarged abdominal lymph nodes with immunohistochemical staining.

Isolated Aplasia Cutis Congenita on Left Foot in Chinese Neonate

Aplasia Cutis Congenita (ACC) is a rare condition characterized by absence of skin layers, usually on the scalp, but can also affect the foot. ACC can occur as an isolated condition or in the presence of the other congenital anomalies. Here we describe a case of a 1-day-old baby boy with an isolated ACC of the left foot, with no family or siblings positive disease history. The patient was managed by both conservatively and surgically until the defect has formed scar tissue 5 months later.

Total segmental aplasia of uterus body in bitch

Congenital anomalies of the female tubular genital tract are rare and known as segmental aplasia, resulting from incomplete development of paramesonephric ducts during embryonic stage, which originate from the cranial portion of vagina, cervix, uterus and oviducts. In the literature consulted, several reports of uterine horn aplasia are described in bitches, however there is only one report described in the world literature on total aplasia of the body of the uterus. This study aimed to describe the case of a two years old bitch, no breed defined, with 12 kg of weight, with segmental aplasia of uterus body diagnosed during ovariohysterectomy. During surgery it was noted that the bitch had the ovaries and uterine horns in their anatomical position, however an aplasia was noted in the body of the uterus. It was also found that the uterine horns had liquid inside, suggesting hydrometra. It was concluded in this case that although segmental aplasia of the body of the uterus is rare and difficult to diagnose by maintaining normal cyclicity of the female, it should be considered in dogs that do not have bloody vaginal discharge in proestrus and can not become pregnant after natural coverage or artificial insemination.

Gingival enlargement induced by cyclosporine in Medullary aplasia:A case report

BACKGROUND Cyclosporine is an immunosuppressive agent used effectively for treatment of a rare haematological disorder known as medullary aplasia.This drug prevents several side effects,including gingival enlargement(GE)which compromises aesthetics,phonetics and chewing,and also predisposes patients to periodontitis.CASE SUMMARY This clinical case reports a 41-year-old woman who presented with cyclosporineinduced GE with underlying periodontitis and medullary aplasia.The management of the disease was approached through multidisciplinary strategy which allowed for accurate diagnosis and a strategic treatment based on the systemic condition and severity of oral pathology.The diagnosis was confirmed through histopathological analysis.The treatment was carried out in phases:Initial(oral hygiene motivation,mechanical supragingival plaque control,and non-surgical therapy);systemic treatment,corrective treatment,and maintenance.CONCLUSION Multidisciplinary management of cyclosporine-induced GE and medullary aplasia allows for correct diagnosis and effective treatment of this pathological expression through a phased therapeutic approach.

Different Clinical Phenotypes in Adams-Oliver Syndrome Conservative Approach to Aplasia Cutis: A Report of Two Cases

Adams-Oliver Syndrome (AOS) is a rare genetic disease characterized by combination of aplasia cutis congenita (ACC) and terminal transverse limb defects (TTLD), often accompanied by defects in scalp and skull ossification. Different clinical phenotypes may be related to variable severity both of aplasia cutis and TTLD, and of minor clinical features as cutis marmorata telangiectatica congenita (CMTC), congenital cardiac defect and vascular anomalies. The treatment is multidisciplinary: dermatologic, orthopedic and surgical consult should be required. It still remains unclear how to treat patients with a large skin defect that can‘t be closed primarly and may require both surgical and conservative management. We report two cases of AOS with typical limb defects and an area of aplasia cutis over vertex of the scalp managed conservatively with two different dermatologic devices.

Treatment for CD57-negativeγδT-cell large granular lymphocytic leukemia with pure red cell aplasia:A case report

BACKGROUND T-cell large granular lymphocytic leukemia(T-LGLL)is a rare type of aplastic anemia with diverse clinical manifestations.Concomitant diseases are often present at the first manifestation.We describe the treatment of a patient with CD57-negativeγδT-LGLL with pure red cell aplasia(PRCA).CASE SUMMARY A 34-year-old woman with a 20-year history of anemia visited our hospital owing to severe dizziness and was admitted.Her condition was diagnosed as CD57-negativeγδT-LGLL with PRCA through bone marrow cytology,bone marrow pathology,bone marrow flow cytometry,bone marrow multiplex polymerase chain reaction combined with fluorescent fragment analysis,and other tests.Treatment with prednisone,methotrexate,and subcutaneous erythropoietin did not significantly change her hemoglobin level.After treatment with oral cyclophosphamide for 3 mo,her hemoglobin level increased to approximately 100 g/L.After 5 mo of treatment,the patient could perform activities of daily living independently.CONCLUSION The treatment of CD57-negativeγδT-LGLL with PRCA with cyclophosphamide helps to improve prognosis.

Angioimmunoblastic T-cell lymphoma accompanied by pure red cell aplasia:A case report

BACKGROUND Angioimmunoblastic T-cell lymphoma(AITL)is a peripheral T-cell lymphoma,which is a rare subtype of lymphoma.Patients with AITL often have skin lesions,which are observed in 50%of all cases;the chief complaint of this patient was palpable purpura.AITL often complicates autoimmune or hematological disorders;however,among these,pure red cell aplasia(PRCA)is a very rare complication of AITL.We herein report a case of AITL with PRCA.CASE SUMMARY A 77-year-old Japanese man presented to our hospital with complaints of loss of appetite for 2 mo and a 10-d history of palpable purpura.On physical examination,the patient was afebrile but had bilateral multiple palpable purpuric lesions over the lower extremities,lower abdomen,and part of the upper extremities.Moreover,lymphadenopathy of the bilateral inguinal,cervical,and supraclavicular nodes was noted.Laboratory and imaging studies and skin biopsy were conducted but were inconclusive.Based on inguinal lymph node excisional biopsy,we diagnosed the patient with AITL.Subsequently,the patient developed progressive normocytic normochromic anemia that necessitated almost daily blood transfusion.The clinical presentations and results of bone marrow assessment were consistent with those of PRCA,which is associated with AITL.Chemotherapy was initiated but was not effective.The patient refused further chemotherapy and opted to continue receiving best supportive care.CONCLUSION PRCA is an extremely rare complication of AITL.As the pathophysiology remains unclear,further research is warranted.

A Case of Coexistence of Aplasia Cutis Congenita and Giant Congenital Melanocytic Nevus:Coexistence of Two Rare Skin Diseases

Aplasia cutis congenita(ACC)is a rare disease that is characterized by complete or partial absence of skin at birth,either in a localized or widespread region.Melanocytic nevi refers to tumor-like malformations of the skin or mucous membrane caused by benign proliferation of melanocytes.It is classified as a giant congenital melanocytic nevus(GCMN)when the diameter of the largest nevus exceeds 20 cm.The co-occurrence of ACC and GCMN is extremely rare,to the best of our knowledge.We report a case of coexistence of ACC and GCMN of infancy in a 2-month-old male infant.The lesions consisted of a large hyperpigmented plaque occupying most of the trunk and pelvic region,and smaller hyperpigmented plaques on the trunk,head,and extremities.Additionally,there were large,sharply marginated,triangular,depressed atrophic plaques covered by thin,translucent,glistening epithelial membranes in the center of the GCMN on the back.The presumptive diagnosis was coexistence of GCMN and ACC.This could be a manifestation of SCALP syndrome,a rare neuro-cutaneous condition characterized by the presence of Sebaceous nevus,Central nervous system(CNS)malformations,Aplasia cutis congenita,Limbal dermoid and Pigmented(giant melanocytic)nevus.

Pure Red Cell Aplasia Caused by Parvovirus B19 in Patients with Human Immunodeficiency Virus Infection: A Series of Four Cases

Parvovirus B19(B19V)infection can cause pure red cell aplasia(PRCA)in patients with human immunodeficiency virus(HIV)infection.Intravenous immunoglobulin(IVIG)is a preferred treatment option.From July 2019 to March 2022,four patients with HIV infection were admitted to Guangzhou Eighth People’s Hospital with dizziness and fatigue and were diagnosed with PRCA.Blood investigations revealed severe anemia and the B19V genome.Therefore,the four patients were diagnosed with B19V-induced PRCA.All four patients received red blood cell transfusion in the setting of antiretroviral therapy,and two of the four patients received intravenous immunoglobulin(IVIG).After 3-7 months of treatment,all four patients recovered,although two did not receive IVIG.This suggests that IVIG is not always necessary for the treatment of PRCA in patients with HIV infection and that effective antiretroviral therapy and immunological reconstitution play an important role in the eradication of parvovirus.

Acquired pure red cell aplasia:unraveling the immune pathogenesis

Acquired pure red cell aplasia(aPRCA)is a rare hematological disorder characterized by normochromic,normocytic anemia,reticulocytopenia,and the absence of erythroblasts.The pathogenesis of aPRCA has remained elusive.This review delves into the intricate web of immune mechanisms underlying the development of this enigmatic condition.By exploring immune responses,cytotoxic effects,and antibody-mediated processes,we dissect the immune-driven assault on erythroid progenitors.The classification of aPRCA,including its primary and secondary forms,is elucidated,with a particular emphasis on etiological factors such as viruses,drugs,thymoma,and large granular lymphocytic leukemia.Furthermore,we discuss the implications of cytogenetic changes in erythroid progenitors and immune cells in the pathophysiology of aPRCA.This comprehensive overview aims to shed light on the complex interplay between immune dysregulation and erythroid failure in aPRCA,offering insights that will be crucial for better understanding and treating this disease.

儿童CD19 CAR-T细胞治疗相关B细胞再生障碍的临床意义和对策

急性B系淋巴细胞白血病(B-ALL)患儿在CD 19 CAR-T细胞治疗后普遍发生B细胞再生障碍(BCA),BCA持续的时间长短对患者的免疫状态及预后会产生影响。对BCA的充分认识有助于临床医师科学、规范、合理地选择治疗策略,减少CAR-T治疗后白血病患儿的感染机会,提高生活质量,改善预后。