Acute liver failure and aplastic crisis due to anorexia nervosa in an adolescent girl: a case report.

Anorexia nervosa (AN) has a high mortality rate due to the widespread organ dysfunction caused by the underlying severe malnutrition. Malnutrition-induced hepatitis is common among individuals with AN especially as body mass index decreases, while acute liver failure and aplastic crisis related to coagulation disease and encephalopathy rarely occur in AN patients. The supervised increase of caloric intake can quickly improve the elevated aminotransferases caused by starvation and aplastic crisis. This current case report describes a 12-year-old adolescent girl who was admitted with a 3-month history of weight loss. Within 3 months, she had lost 10 kg of weight. The girl was diagnosed with AN, acute liver failure, severe malnutrition with emaciation, electrolyte disorder, bradycardia and aplastic crisis. She was gradually supplemented with vitamins and enteral nutrition to avoid refeeding syndrome. After treatment, her liver function and haematopoietic function returned to normal. In conclusion, acute liver failure and aplastic crisis are rare but potentially life-threatening complications of AN, which could be improved by supervised feeding and timely rehydration. AN should be considered as the potential aetiology of acute liver failure and aplastic crisis.

Tuberculosis-induced aplastic crisis and atypical lymphocyte expansion in advanced myelodysplastic syndrome: A case report and review of literature.

BACKGROUND: Myelodysplastic syndrome (MDS) is caused by malignant proliferation and ineffective hematopoiesis. Oncogenic somatic mutations and increased apoptosis, necroptosis and pyroptosis lead to the accumulation of earlier hematopoietic progenitors and impaired productivity of mature blood cells. An increased percentage of myeloblasts and the presence of unfavorable somatic mutations are signs of leukemic hematopoiesis and indicators of entrance into an advanced stage. Bone marrow cellularity and myeloblasts usually increase with disease progression. However, aplastic crisis occasionally occurs in advanced MDS. CASE SUMMARY: A 72-year-old male patient was definitively diagnosed with MDS with excess blasts-1 (MDS-EB-1) based on an increase in the percentages of myeloblasts and cluster of differentiation (CD)34+ hematopoietic progenitors and the identification of myeloid neoplasm-associated somatic mutations in bone marrow samples. The patient was treated with hypomethylation therapy and was able to maintain a steady disease state for 2 years. In the treatment process, the advanced MDS patient experienced an episode of progressive pancytopenia and bone marrow aplasia. During the aplastic crisis, the bone marrow was infiltrated with sparsely distributed atypical lymphocytes. Surprisingly, the leukemic cells disappeared. Immunological analysis revealed that the atypical lymphocytes expressed a high frequency of CD3, CD5, CD8, CD16, CD56 and CD57, suggesting the activation of autoimmune cytotoxic T-lymphocytes and natural killer (NK)/NKT cells that suppressed both normal and leukemic hematopoiesis. Elevated serum levels of inflammatory cytokines, including interleukin (IL)-6, interferon-gamma (IFN-γ) and tumor necrosis factor-alpha (TNF-α), confirmed the deranged type I immune responses. This morphological and immunological signature led to the diagnosis of severe aplastic anemia secondary to large granule lymphocyte leukemia. Disseminated tuberculosis was suspected upon radiological examinations in the search for an inflammatory niche. Antituberculosis treatment led to reversion of the aplastic crisis, disappearance of the atypical lymphocytes, increased marrow cellularity and 2 mo of hematological remission, providing strong evidence that disseminated tuberculosis was responsible for the development of the aplastic crisis, the regression of leukemic cells and the activation of CD56+ atypical lymphocytes. Reinstitution of hypomethylation therapy in the following 19 mo allowed the patient to maintain a steady disease state. However, the patient transformed the disease phenotype into acute myeloid leukemia and eventually died of disease progression and an overwhelming infectious episode. CONCLUSION: Disseminated tuberculosis can induce CD56+ lymphocyte infiltration in the bone marrow and in turn suppress both normal and leukemic hematopoiesis, resulting in the development of aplastic crisis and leukemic cell regression.

Erythema Infectiosum: A Narrative Review.

BACKGROUND: Erythema infectiosum occurs worldwide. School-aged children are most often affected. Since the diagnosis is mainly clinical, physicians should be well-versed in the clinical manifestations of erythema infectiosum to avoid misdiagnosis, unnecessary investigations, and mismanagement of the disease. OBJECTIVE: The purpose of this article is to familiarize physicians with the wide spectrum of clinical manifestations and complications of erythema infectiosum associated with parvovirus B19 infection. METHODS: A search was conducted in July 2022 in PubMed Clinical Queries using the key terms " Erythema infectiosum" OR "Fifth disease" OR "Slapped cheek disease". The search strategy included all clinical trials, observational studies, and reviews published within the past 10 years. Only papers published in the English literature were included in this review. The information retrieved from the above search was used in the compilation of the present article. RESULTS: Erythema infectiosum is a common exanthematous illness of childhood caused by parvovirus B19. Parvovirus B19 spreads mainly by respiratory tract secretions and, to a lesser extent, the saliva of infected individuals. Children between 4 and 10 years of age are most often affected. The incubation period is usually 4 to 14 days. Prodromal symptoms are usually mild and consist of low-grade fever, headache, malaise, and myalgia. The rash typically evolves in 3 stages. The initial stage is an erythematous rash on the cheeks, with a characteristic "slapped cheek" appearance. In the second stage, the rash spreads concurrently or quickly to the trunk, extremities, and buttocks as diffuse macular erythema. The rash tends to be more intense on extensor surfaces. The palms and soles are typically spared. Central clearing of the rash results in a characteristic lacy or reticulated appearance. The rash usually resolves spontaneously within three weeks without sequelae. The third stage is characterized by evanescence and recrudescence. In adults, the rash is less pronounced than that in children and is often atypical. Only approximately 20% of affected adults have an erythematous rash on the face. In adults, the rash is more frequently found on the legs, followed by the trunk, and arms. A reticulated or lacy erythema is noted in 80% of cases which helps to distinguish erythema infectiosum from other exanthems. Pruritus is noted in approximately 50% of cases. The diagnosis is mainly clinical. The many manifestations of parvovirus B19 infection can pose a diagnostic challenge even to the best diagnostician. Complications include arthritis, arthralgia, and transient aplastic crisis. In most cases, treatment is symptomatic and supportive. When parvovirus B19 infection occurs in pregnant women, hydrops fetalis becomes a real concern. CONCLUSION: Erythema infectiosum, the most common clinical manifestation of parvovirus B19 infection, is characterized by a "slapped cheek" appearance on the face and lacy exanthem on the trunk and extremities. Parvovirus B19 infection is associated with a wide spectrum of clinical manifestations. Physicians should be aware of potential complications and conditions associated with parvovirus B19 infection, especially in individuals who are immunocompromised, chronically anemic, or pregnant.

Valproic acid induced aplastic crisis and Stevens-Johnson syndrome in a single pediatric patient.

Valproic acid (VPA) is a commonly used antiepileptic drug (AED). Aplastic crisis is defined as acute arrest of hematopoiesis. Stevens-Johnson syndrome (SJS) is a fatal cutaneous adverse drug reaction. We herein report a rare case of aplastic crisis and SJS in a single pediatric patient that were probably caused by VPA. A 2-year-old girl was involved in a car accident. She was diagnosed with skull fractures, cerebral contusions, pulmonary contusions, and fractures of the left iliac bone by computed tomography. VPA was administered as prophylaxis for post-traumatic epilepsy. From day 13, she developed repeated high fevers, and multiple antibiotics were ineffective; she was then transferred to our pediatric intensive care unit. After transfer, she developed liver function impairment, decreased peripheral blood cell counts, and skin damage. After withdrawal of the VPA and administration of prednisone, intravenous immunoglobulin, local skin care, and nutritional support, her body temperature normalized and her hematopoietic function and skin lesions successively resolved. She was transferred out of the pediatric intensive care unit on day 56 and discharged on day 70. At the 6-month follow-up, a blood examination was normal, and repeat computed tomography revealed multiple softening foci of the bilateral brain and less subdural effusion than before. To our knowledge, no report to date has described aplastic crisis and SJS in a single patient. The purpose of this paper is to increase clinicians' knowledge in the treatment of adverse drug reactions (ADRs) and emphasize the importance of standardized application and strict monitoring of VPA in patients with post-traumatic brain trauma.

Hemophagocytic lymphohistiocytosis associated with parvovirus B19-induced aplastic crisis in a hereditary spherocytosis patient: A case report and literature review.

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation. It occurs because of severe inflammation due to uncontrolled proliferation of activated lymphocytes and histiocytes, characterized by the production of excessive levels of cytokines. Virus-associated HLH is a well-known entity, and parvovirus B19 is one of the common causes. Parvovirus B19 can also affect blood cell lineages. Therefore, HLH may be accompanied by several diseases such as cytopenia, aplastic anemia, and myelodysplastic syndrome. Herein, we report the case of a patient with hereditary spherocytosis who was diagnosed with parvovirus B19-induced HLH and aplastic crisis. A 7-year-old girl presented to our hospital with fever, pleural effusion, pancytopenia, hepatosplenomegaly, and hypotension. A bone marrow biopsy was performed under the suspicion of HLH, which revealed hemophagocytes. The diagnostic criteria for HLH were met, and prompt chemoimmunotherapy was initiated considering the clinically unstable situation. Her health improved rapidly after initiating treatment. Further study revealed that she had hereditary spherocytosis, and parvovirus B19 had caused aplastic crisis and HLH. The patient's clinical progress was excellent, and chemoimmunotherapy was reduced and discontinued at an early stage. This case shows that aplastic crisis and HLH can coexist with parvovirus B19 infection in patients with hereditary spherocytosis. Although the prognosis was good in this case of HLH caused by parvovirus B19, early detection and active treatment are essential.

A Case with Iron Deficiency Anemia Developed Aplastic Crisis.

A toddler with an unbalanced diet and gastrointestinal bleeding by juvenile polyp developed an aplastic crisis due to the human parvovirus B19 (HPVB19). Although he exhibited microcytic anemia without iron deficiency in the acute phase of HPVB19 infection, he presented with iron deficiency anemia (IDA) in the chronic phase. IDA results in erythroblast hyperplasia and shortened red blood cell lifespan as like congenital hemolytic diseases, which may lead to an aplastic crisis during HPVB19 infection. It should be noted that iron deficiency is often masked, and microcytic anemia may be a clue for IDA.

Disseminated Human Parvovirus B19 Infection Induced Multiple Organ Dysfunction Syndrome in an Adult Patient With Alcoholic Hepatitis Complicated by Hemolytic Anemia: A Case Report and Literature Review.

Background: Human parvovirus B19 (B19) can cause acute hepatitis and is attributed to the high mortality of alcoholic hepatitis (AH). B19 infection is generally self-healing in previously healthy people, but it can cause fatal effects in some high-risk groups and increase its virulence and infectivity. Disseminated B19 infection-induced multiple organ dysfunction syndrome (MODS) in patients with AH has not been reported yet. Here, we described B19 viremia in an adult patient with AH accompanied by hemolytic anemia (HA), leading to disseminated infection and secondary MODS, as well as self-limiting B19 infections in seven nurses caring for him. Meanwhile, we reviewed the literature on AH and B19 infection. Case Presentation: A 43-year-old male patient with AH accompanied by HA was transferred to the Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China, on March 31, 2021. After supportive treatment, his transaminase and bilirubin levels were reduced, but his anemia worsened. He received a red blood cell (RBC) infusion on April 9 for hemoglobin (Hb) lower than 6 g/dl. On April 13, he suddenly had a high fever. Under empirical anti-infection, his high fever dropped and maintained at a low fever level; however, his anemia worsened. On April 25, he was transferred to the medical intensive care unit (MICU) due to severe pneumonia, acute respiratory distress syndrome (ARDS), acute aplastic crisis (AAC), and hemophagocytic syndrome (HPS), which were subsequently confirmed to be related to B19 infection. After methylprednisolone, intravenous immunoglobulin (IVIG), empirical anti-infection, and supportive treatment, the lung infection improved, but hematopoietic and liver abnormalities aggravated, and systemic B19 infection occurred. Finally, the patient developed a refractory arrhythmia, heart failure, and shock and was referred to a local hospital by his family on May 8, 2021. Unfortunately, he died the next day. Fourteen days after he was transferred to MICU, seven nurses caring for him in his first two days in the MICU developed self-limiting erythema infectiosum (EI). Conclusions: B19 infection is self-limiting in healthy people, with low virulence and infectivity; however, in AH patients with HA, it can lead to fatal consequences and high contagion.

Novel Surrogate Neutralizing Assay Supports Parvovirus B19 Vaccine Development for Children with Sickle Cell Disease.

Children with sickle cell disease (SCD) suffer life-threatening transient aplastic crisis (TAC) when infected with parvovirus B19. In utero, infection of healthy fetuses may result in anemia, hydrops, and death. Unfortunately, although promising vaccine candidates exist, no product has yet been licensed. One barrier to vaccine development has been the lack of a cost-effective, standardized parvovirus B19 neutralization assay. To fill this void, we evaluated the unique region of VP1 (VP1u), which contains prominent targets of neutralizing antibodies. We discovered an antigenic cross-reactivity between VP1 and VP2 that, at first, thwarted the development of a surrogate neutralization assay. We overcame the cross-reactivity by designing a mutated VP1u (VP1uAT) fragment. A new VP1uAT ELISA yielded results well correlated with neutralization (Spearman's correlation coefficient = 0.581; p = 0.001), superior to results from a standard clinical diagnostic ELISA or an ELISA with virus-like particles. Virus-specific antibodies from children with TAC, measured by the VP1uAT and neutralization assays, but not other assays, gradually increased from days 0 to 120 post-hospitalization. We propose that this novel and technically simple VP1uAT ELISA might now serve as a surrogate for the neutralization assay to support rapid development of a parvovirus B19 vaccine.

[Persistent anemia after kidney transplantation in a 36-year-old male patient-an unusual cause]. / Persistierende Anämie nach Nierentransplantation bei einem 36-jährigen Patienten ­ eine ungewöhnliche Ursache.

An allogeneic kidney transplantation (match 1­1­0, cytomegalovirus, CMV, donor, D, +/recipient, R, - high risk) was performed in a 36-year-old patient. The patient was on dialysis due to a tubulointerstitial nephritis confirmed by biopsy 11 years previously. Posttransplantation there was a gradual decrease in the hemoglobin (Hb) level from 11.4 g/dl to 7.3 g/dl during the initial hospitalization period. Initially this was explained by the kidney transplantation and chronic fibrosing antral gastritis with erosions. Despite repeated transfusion of red cell concentrates, a refractory anemia persisted, which is why the patient presented several times at our clinic for further diagnosis and treatment. The presence of giant erythroblasts in the bone marrow and quantitative detection of parvovirus B19 (>900 million IU/ml DNA replications) was consistent with a virus-associated red cell aplasia. Intravenous immunoglobulin administration was established and showed long-term therapeutic success.

Hiperplasia eritroblástica secundaria a infección aguda por parvovirus B19

Resumen La infección aguda por parvovirus B19 es una enfermedad autolimitada en pacientes sin trastornos inmunitarios. Sin embargo, en pacientes con discrasias sanguíneas pueden manifestarse con una crisis aplásica. Presentamos el caso de un varón de 48 años, con una esferocitosis hereditaria no diagnosticada previamente, la cual debutó con una crisis aplásica inducida por una infección aguda de parvovirus B19. La sospecha clínica se planteó luego del análisis histopatológico de la médula ósea, en el que se observó una hiperplasia eritroblástica, con precursores eritroides gigantes e inclusiones nucleares virales, y cuyo análisis inmunohistoquímico fue positivo para la proteína de la cápside viral VP1 y VP2 de parvovirus B19 en células infectadas. Se confirmó la sospecha diagnóstica con la detección de anticuerpos IgM de parvovirus B19. De acuerdo a nuestra revisión, este es el primer reporte de un adulto en Latinoamérica que debutó con una crisis aplásica inducida por una infección aguda por parvovirus B19, como primera manifestación de una esferocitosis hereditaria.

Abstract Acute parvovirus B19 infection is a self-limiting disease in patients with normal immune response. However, in patients with blood dyscrasias, it is possible to present with an aplastic crisis. We present the case of a 48-year-old man who had developed an aplastic crisis as a result of an acute parvovirus B19 infection with an undiagnosed hereditary spherocytosis. Suspicions of the parvovirus infection began to arise after a routine bone marrow histopathological analysis which showed erythroblastic hyperplasia with giant erythroid precursor and viral inclusions. A subsequent immunohistochemical analysis tested positive for VP1 and VP2 capsid proteins of parvovirus B19 in infected cells. The diagnostic suspicion was later confirmed with the presence of anti-parvovirus B19 IgM. According to our review, this is the first published case in Latin America that documents an adult patient with normal immune response whose first symptom of hereditary spherocytosis was an aplastic crisis induced by an acute parvovirus B19 infection.

Parvovirus-Induced Transient Aplastic Crisis in a Patient With Newly Diagnosed Hereditary Spherocytosis.

Parvovirus B19 infections are prevalent in children and commonly present as slapped cheek fever, also known as the fifth disease. They are seen frequently in daycares and professions that require close contact with children. The most common presentation is a rash that is prominent on the cheeks; less common symptoms include painful or swollen joints (polyarthopathy syndrome). The infection is self-limited and resolves within one to two weeks. The virus has an affinity to the red blood cell (RBC) precursors and can rarely cause temporary cessation of the bone marrow's RBC production, leading to aplastic anemia. This is especially of significance in patients predisposed to increased RBC destruction, such as hereditary spherocytosis, sickle cell anemia, and other morphological abnormalities of the RBC. The overlapping arrest of RBC production and excessive destruction leads to a transient aplastic crisis (TAC), leading to severe life-threatening anemia, requiring blood urgent blood transfusions. There have been many studies reporting the incidence of TAC in patients with sickle cell crisis. Only a few cases have been reported in patients with hereditary spherocytosis.

Human parvovirus B19 infection in patients with or without underlying diseases.

BACKGROUND/PURPOSE: The clinical presentations of parvovirus B19 in patients with underlying diseases have greater diversity than previously healthy patients. We retrospectively identified patients with polymerase chain reaction (PCR)-confirmed parvovirus B19 infection in attempt to describe its clinical features especially in these populations. METHODS: From 2009 to 2018, patients with real-time PCR-confirmed parvovirus B19 infection were collected. Comparisons were done between previously healthy patients and patients with preexisting diseases, as well as patients with high (>5.5 × 105 copies/mL sera) and low viral loads. RESULTS: Parvovirus B19 DNA was detected in 31 patients. Fourteen (45%) patients had underlying diseases, including six (19%) with immunologic diseases, five (16%) with hematologic diseases, and three (10%) with cardiopulmonary diseases. Only seven (23%) patients received an initial impression of erythema infectiosum prior to positive PCR. A higher proportion of patients with underlying diseases presented with fatigue and pallor, and suffered from tachycardia and hepatosplenomegaly compared to previously healthy patients. Among patients with a high viral load, a substantial proportion were of older age, suffered fatigue, and anemia. There was a trend of patients with immunologic comorbidity having a higher viral load. CONCLUSION: The classical parvovirus B19 manifestations were less frequently observed in patients with a preexisting disease compared with previously healthy patients. Depending on host factors, the symptoms of parvovirus B19 infection can be multifaceted.

A Differential Approach to an Uncommon Case of Acute Anemia in a Child With Sickle Cell Disease.

Introduction: Hyperhemolytic crisis is a rare and dangerous complication of sickle cell disease where the hemoglobin level drops rapidly. This can quickly lead to organ failure and death. In the literature, most cases of hyperhemolysis in sickle cell patients followed a red cell transfusion. Case Summary: In this article, we report a case of a 6-year-old African American boy with sickle cell disease who presented with fever, increased work of breathing, and consolidation in the left lower lobe of the lung on chest X-ray. He initially improved with oxygen, fluids, and antibiotics but his hemoglobin acutely dropped from 7.6 to 6 g/dL the next day of admission. He was not previously transfused, and his reticulocyte count remained high. Subsequent transfusion recovered his hemoglobin. Conclusion: This case demonstrates that in the background of the chronic hemolysis of sickle cell disease, an acute anemia should warrant exploration of aplastic crisis (parvovirus infection), immune hemolytic anemia, hepatic sequestration crisis, splenic sequestration crisis, and hyperhemolytic crisis as possible etiologies. Ongoing reticulocytosis and a source of infection may direct suspicion especially toward hyperhemolytic crisis even without preceding red cell transfusion. We propose that the optimum management should include full supportive care (including transfusions if necessary) and treatment of the underlying cause of hemolysis (such as infections or drug exposure).

Anemia hemolítica autoinmune por crioaglutininas y crisis aplásica en una paciente con hemoglobinopatía SC / Autoimmune hemolytic anemia with cryoagglutinins and aplastic crisis in a patient with SC hemoglobinopathy

La infección por parvovirus B19 humano, es la causa de la mayor parte de los casos de crisis aplásica transitoria que aparecen de forma brusca en pacientes con enfermedades hemolíticas crónicas, como es el caso de la drepanocitosis. Por otra parte, se han descrito unos pocos casos de infección aguda, por parvovirus B19 humano como causa de anemia hemolítica autoinmune, por medio de la formación de anticuerpos dirigidos contra los glóbulos rojos. La asociación entre drepanocitosis y anemias hemolíticas autoinmunes es poco frecuente. Se reporta un caso poco usual de una paciente adulta, con antecedentes de hemoglobinopatía S/C que presentó una crisis aplásica y posteriormente apareció una anemia hemolítica autoinmune diagnosticada en el Instituto de Hematología e Inmunología. Se trató con dosis inmunosupresoras de esteroide, con lo que se alcanzó la remisión de la anemia hemolítica autoinmune(AU)

Infection with human B19 parvovirus is the cause of most cases of transient aplastic crisis that appear in patients with chronic hemolytic diseases, as in the case of sickle cell disease. On the other hand, a few cases of acute infection by human parvovirus B19 have been described as a cause of autoimmune hemolytic anemia, through the formation of antibodies directed against red blood cells. The association between sickle cell disease and autoimmune hemolytic anemia is rare. We report an unusual case of an adult patient, with a history of S C hemoglobinopathy who presented an aplastic crisis and subsequently an autoimmune hemolytic anemia diagnosed at the Institute of Hematology and Immunology, treated with high steroids doses, reaching the remission of autoimmune hemolytic anemia and constitutes the first report in Cuba(AU)

Rapid Fire: Sickle Cell Disease.

Emergency providers are likely to encounter sickle cell disease-related emergencies. The pathophysiology of emergent complaints are usually related to either an acute anemia or a vasoocclusive crisis. Differentiating between the two is the first step in the workup. Anemic crises must then be differentiated by the source. Vasoocclusive crises must be appropriately treated with aggressive pain management, gentle hydration, and other appropriate adjuncts. Early recognition and treatment are key in providing excellent emergency care to those with sickle cell disease.

Double whammy- acute splenic sequestration crisis in patient with aplastic crisis due to acute parvovirus infection.

Splenic dysfunction is a major feature of sickle cell disease (SCD) and can manifest as acute splenic sequestration crisis (ASSC), which is the earliest life-threatening complication seen in patients with SCD. Aplastic crisis is another potentially deadly complication of sickle cell disease that develops when erythrocyte production temporarily drops. Infection with parvovirus B-19 frequently causes aplastic crises. These two complications are known to be mutually exclusive due to their classic presentation signs and symptoms but there have been few cases where a patient can have concomitant presentation of both phenomena, which can result in a fatal outcome. These few cases force us to rethink the etiology and subsequent management guidelines of these complications. We present to you a case of an unfortunate 23-year-old female who had both complications occurring at the same time, resulting in death.