Influenza B Virus Triggering Macrophage Activation Syndrome in an Infant.

Macrophage activation syndrome (MAS) is a potentially fatal complication caused by excessive activation and expansion of macrophages and T lymphocytes. It can be triggered by various infections and is characterized by the development of cytopenias, hyperferritinemia, liver dysfunction, and coagulopathy. We report a 10-month-old female infant with fever, convulsions, and hepatosplenomegaly. Laboratory data of bicytopenia, low erythrocyte sedimentation rate, and elevated liver enzymes suggested MAS. This was supported by the presence of hyperferritinemia with hypertriglyceridemia. MAS was triggered by influenza B virus. She responded to treatment with immunoglobulin and steroid.

Bicytopenia, especially thrombocytopenia in hemodialysis and non-hemodialysis patients treated with linezolid therapy.

One of the major adverse events associated with linezolid treatment is pancytopenia. However, there are few reports about the tolerability of linezolid among patients undergoing hemodialysis. This study retrospectively investigated the frequency of bicytopenia (thrombocytopenia and erythropenia) secondary to linezolid treatment in patients undergoing and not-undergoing hemodialysis. In total, 181 patients treated with linezolid from January 2010 to July 2012 at Aichi Medical University Hospital were divided into three groups; patients undergoing hemodialysis (HD group), those with creatinine clearance (CLCR) of <50 mL/min (CLCR < 50 group) and those with CLCR of ≥ 50 mL/min (CLCR ≥ 50 group). The incidence of thrombocytopenia, and changes in the platelet (PLT) counts during and after linezolid therapy were compared among three groups. Thrombocytopenia (<75% of the baseline level) occurred in 125 patients (69.1%). PLT reached its nadir 3-4 days after the end of linezolid therapy. In particular, the PLT nadir in HD group occurred earlier than that in non-HD groups (HD, 11.5 days [4-31 days]; CLCR < 50, 14 days [5-43 days]; CLCR ≥ 50, 15.5 days [4-49 days]; p = 0.11). HD group exhibited the greatest rate of reduction of PLT (HD, 24.0% [0.4-93.8%]; CLCR < 50, 23.8% [0.8-92.9%]; CLCR ≥ 50, 22.4% [0.92-92.9%]; p = 0.003). Finally, HD group exhibited the slowest recovery of PLT to its baseline level (HD, 10 days [5-29 days]; CLCR < 50, 9 days [2-16 days]; CLCR ≥ 50, 8 days [3-17 days]; p = 0.09). The incidence of erythropenia was not significantly different among three groups. These results indicate the need to monitor the PLT count during and after linezolid treatment in patients undergoing hemodialysis.

Visceral leishmaniasis misdiagnosed as an upper respiratory infection and iron-deficiency anemia in a 20-month-old male patient: a case report.

BACKGROUND: Visceral Leishmaniasis should be suspected in every patient with a history of splenomegaly, fever, and pancytopenia. It is one of the most dangerous forms of infection and prompt recognition is the key to positive outcome. CASE PRESENTATION: A 20-month-old Caucasian male patient was brought to our hospital as an outpatient with the complaint of persistent fever, which did not improve with empiric antibiotic treatment (> 96 hour after the initial dose). The antibiotic treatment had been prescribed by primary care physician at polyclinic, who also referred the patient to hematologist due to anemia, who prescribed iron supplement. Despite multiple subspecialist visits, bicytopenia was, unfortunately, left unidentified. Upon physical examination no specific signs were detected, however, spleen seemed slightly enlarged. Patient was admitted to the hospital for further work-up, management and evaluation. Abdominal ultrasound, complete blood count and c-reactive protein had been ordered. Hematologist and infectionist were involved, both advised to run serology for Epstein-Barr Virus and Visceral Leishmaniasis. The latter was positive; therefore, patient was transferred to the specialized clinic for specific management. CONCLUSION: Both in endemic and non-endemic areas the awareness about VL should be increased among the medical professionals. We also recommend that our colleagues take the same approach when dealing with bicytopenia and fever, just as with pancytopenia and fever. The medical community should make sure that none of the cases of fever and pancytopenia are overlooked, especially if we have hepatomegaly and/or splenomegaly.

Study of Clinicoetiological Spectrum of Bicytopenia and Pancytopenia in Hospitalized Children.

Background The etiological profile of children with bicytopenia and pancytopenia has a very wide spectrum, ranging from transient causes like infections or nutritional deficiencies to bone marrow failure syndromes. Timely diagnosis and treatment impart favorable prognosis to this entity. There is a paucity of data regarding the etiology of cytopenia in hospitalized children at a tertiary center in India. Additionally, only a few studies have discussed the possible association between the severity of cytopenia at presentation and the possible etiology. Methods This is a cross-sectional observational study analyzing bicytopenia and pancytopenia in hospitalized children. Patient details, along with clinical findings and relevant investigations, were recorded on predesigned pro forma and analyzed statistically. Results Out of 202 children, 174 (86.13%) had bicytopenia, and 28 (13.86%) had pancytopenia, with a male predominance resulting in a male-to-female ratio of 1.65:1. The commonest age group affected was pre-adolescent age group (6-12 years). The causes of bicytopenia and pancytopenia in hospitalized children in the decreasing order of frequency were infections (65.84%), benign hematological disorders (18.81%), systemic illness (10.39%), and malignancies (4.95%). The cytopenia was more severe in children with pancytopenia than bicytopenia. Conclusions Infections outweigh the other causes of bicytopenia and pancytopenia. The severity of the cell line affected can help narrow down a diagnosis of cytopenia etiologies. Most of the children with bicytopenia and pancytopenia had treatable etiology and favorable outcomes.

Unexplained Chronic Liver Disease and Hemolytic Anemia in a Young Girl: A Case of Wilson's Disease.

Wilson's disease (WD) is an autosomal recessive disorder affecting the metabolism of copper that can present with a variety of clinical symptoms. Low levels of serum copper and ceruloplasmin, increased excretion of copper in the urine, and/or increasing quantities of copper in the liver are diagnostic indicators. The gold standard for diagnosis is genetic testing. The care approach includes the utilization of liver transplants as a therapeutic option in advanced patients and the use of copper-chelating medications. We describe a unique case of WD in a 14-year-old girl who presented with ascites, hemolytic anemia, and liver dysfunction. There was no indication of abdominal TB, and her viral, autoimmune, and hemolytic profiles were all normal. Low serum ceruloplasmin, elevated urine copper, and distinctive liver histology all supported the WD diagnosis. After starting penicillamine medication, the patient's symptoms improved, but her blood counts did not. This example emphasizes how crucial it is to rule out WD in patients with chronic liver disease, hemolytic anemia, and unexplained ascites, particularly in younger age groups.

Late Diagnosis of Primary Hyperoxaluria in an Adult Patient With End-Stage Renal Disease and Bicytopenia.

Primary hyperoxaluria (PH) is a rare genetic condition that disrupts the normal process of glyoxylate metabolism, resulting in an overproduction of oxalate. This excessive oxalate production leads to the accumulation of calcium oxalate (known as oxalosis) throughout various organs in the body. The urinary tract, specifically the renal parenchyma, is the first location where the deposition of calcium oxalate begins in PH. These deposits are responsible for nephrocalcinosis and tubule­interstitial nephritis which leads to end­stage renal failure. This is then followed by the accumulation of oxalate in other organs including the bone marrow. Herein, we report the case of a 22-year-old male patient who presented with bicytopenia; he had a history of end-stage renal disease preceded by recurrent urolithiasis and nephrolithiasis episodes since the age of 3 years. A bone marrow biopsy was performed for evaluation of the bicytopenia which led to the diagnosis of PH.

Salmonella Paratyphi and multiorgan dysfunction: A rare case report.

Salmonella typhi (S. typhi) and Salmonella paratyphi A (S. paratyphi A), together known as typhoidal Salmonella, are causal agents for an invasive, serious, and sometimes fatal disease of humans called typhoid fever or paratyphoid fever (also known as enteric fever). S. Typhi, the lineage causing typhoid fever, is the main group; whereas S. Paratyphi A, the lineage causing paratyphoid fever, belongs to the second group, which comprises a set of three paratyphoid types (the other two being S. Paratyphi C and d-tartrate-negative S. Paratyphi B). All these lineages are adapted to humans, with S. Typhi and S. Paratyphi A being strictly restricted to growth in humans, and S. Paratyphi C being able to establish infections in experimental animals quite easily (at moderate infection doses); the host-restriction status of d-tartrate-negative S. Paratyphi B is so far unclear. The potential source of infection is the use of sewage-contaminated water in plants and vegetable irrigation and clinical presentation is varied, mainly presenting with fever, malaise, abdominal discomfort, and nonspecific symptoms often confused with other causes of the febrile syndrome. S. Paratyphi is usually a mild form of disease without any complication, but we report a complicated case of Paratyphi, who presented with fever and gastrointestinal symptoms complicated by multiorgan dysfunction needing mechanical ventilatory support, multiple hemodialysis, and blood transfusion. Fortunately, he recovered from all the insults and was discharged home in stable condition on the 26th day of hospitalization.

Therapeutic Options in Refractory Evans Syndrome: A Case Report.

Evans syndrome is a rare autoimmune disease, characterized by at least two immune cytopenias, most frequently anemia and thrombocytopenia and rarely immune neutropenia. It has a variable clinical presentation and is rarely diagnosed in adults. It can be idiopathic or secondary to lymphoproliferative disease, infections, autoimmune diseases, drugs, and immunodeficiencies in about 50% of cases. It is characterized by a chronic, relapsing, potentially fatal course due to its hemorrhagic complications as well as complications associated with the long-term immunosuppressive treatment required to control the disease, such as infectious diseases, and cardiovascular and renal complications. Its prognosis depends on the underlying cause. Because of its rarity, the treatment is empirical, based mostly on case series and recommendations for the treatment of other immune cytopenias. The underlying disease and demographic characteristics also play an important role in choosing the treatment, which should be adapted individually to each patient. We present a case of an elderly patient with idiopathic autoimmune hemolytic anemia and thrombocytopenia, refractory to various treatment options.

Valproate-Induced Bicytopenia: A Case Study.

This case report describes a 22-year-old male patient diagnosed with schizoaffective disorder. A multidrug regimen including olanzapine and lorazepam was initiated for the patient. Sodium valproate was added to the regimen in due course and it was associated with bicytopenia in the form of thrombocytopenia and leukopenia. Valproate was identified as the offending drug and further doses were withheld. Lithium was used as a substitute to valproate and the patient spontaneously recovered without any further complications. This case report highlights the necessity of periodic investigations and frequent logging of blood indices to counter the threat of fatal adverse drug reactions.

Hepatosplenic T Cell Lymphoma: Diagnostic Conundrum.

Hepatosplenic T cell lymphoma (HSTCL) is a very rare and aggressive peripheral T cell lymphoma that comprises less than 1% of Non-Hodgkin lymphomas (NHL). It is derived from cytotoxic T-cells, usually of γδ T cell receptor type, and is characterized by primary extranodal disease with typical sinusoidal infiltration of the liver, spleen and bone marrow by medium-sized lymphoid cells. HSTCL occurs more frequently in immunocompromised patients, especially in those receiving long-term immunosuppressive therapy. The differential diagnosis is varied, and the clinical course is dismal with a poor response to currently available therapies. Herein we report a case of HSTCL in a 20-year-old immunocompetent male who presented with fever, pallor, weight loss, bicytopenia, hepatomegaly, and massive splenomegaly, highlighting the diagnostic conundrum and pointers towards an accurate diagnosis. The key role for diagnosis was the combination of morphologic finding of atypical lymphoid cells in the bone marrow, typical immunophenotypic profile on flow cytometry and the pattern of involvement of the liver and the spleen, even in the absence of full-fledged diagnostic panels and tools. The report of this case is an endeavor to emphasize the high index of suspicion for timely detection of such a rare entity.

Bicytopenia revealing SARS-Cov-2 pneumonia in a 4 month old infant: A case report.

Background. Introduction: Only a few cases of SARS-CoV-2 pneumonia in infants have been reported, and the epidemiological, clinical presentation and the course of these patients are not yet fully understood. Clinical presentation: we report the case of a 4-month-old infant admitted to the intensive care unit for the management of a hemorrhagic syndrome which turned out to be Sars cov 2 pneumonia. Discussion: While research into the COVID-19 pandemic is still ongoing, it appears that young children are less likely to be infected with SARS-CoV-2 and their infection is less severe. Conclusion: SARS-CoV-2 pneumonia would be less frequent and less serious in infants, but this should not make us omit this diagnosis in the face of acute respiratory distress, especially if there is an underlying family cluster.

Pregnancy-associated aplastic anemia: a case-based review.

Introduction: Pregnancy-associated aplastic anemia (pAA) occurs when aplastic anemia (AA) is diagnosed for the first time during pregnancy and it is a rare but serious condition leading to severe maternal and fetal complications. It is unknown whether pregnancy triggers bone marrow failure or if this state is unrelated to the pathogenesis of pAA.Areas covered: In this review, three new cases of pAA are presented and its controversial etiologic relationship with pregnancy, its atypical presentation, and management are also discussed. Furthermore, a literature review of pAA cases between 1975 and 2020 was performed in PubMed, accessed via the National Library of Medicine PubMed interface. Keywords included 'aplastic anemia' AND 'pregnancy'. We found 54 cases reported in the literature with a clear diagnosis of pAA.Expert opinion: The diagnosis of pAA is challenging since pregnancy is associated with physiologic hematological changes in the complete blood count which can mask the disease. Meticulous monitoring and adequate support therapy given by a trained multidisciplinary team have the potential to improve outcomes for women and their neonates. All women should receive frequent assessments to optimize their care during pregnancy and after delivery, definitive treatment should be offered.

Comparison of the severity of cytopenias with etiologic factors in patients with pancytopenia and bicytopenia.

INTRODUCTION: The aim of this study was to evaluate the severity of hematological findings according to etiology in patients with pancytopenia and bicytopenia. METHODS: Patients with bicytopenia and pancytopenia who were examined in Diyarbakir Children Hospital Pediatric Hematology and Oncology clinic between June 2017-June 2018 were evaluated retrospectively. RESULTS: Of the 130 patients included in the study, 73 (56.2%) were male and 43 (43.8%) were female. The mean age of the patients was 4.9 ± 4.86. Forty-five (34.6%) patients had pancytopenia and 85 (65.3%) had bicytopenia. The youngest patient was 1-month old and the oldest patient was 18-year-old. The mean blood count parameters were white blood cell (WBC): 10.207 ± 39.781, neutrophil: 1515 ± 1418, hgb: 9.3 ± 2.3, mean corpuscular volume (MCV): 80 ± 13.1, platelet: 118.823 ± 93.645. Three out of 130 patients had hyperleukocytosis (WBC > 50.000/mm3). Vitamin B12 deficiency was detected in 35 patients. When patients with primary hematological disease were compared with patients with secondary causes of cytopenias, a significant difference was found in terms of leukocyte count, hemoglobin level, MCV elevation, and low platelet count. CONCLUSION: The determination of the severity of cytopenias in differential diagnosis may be useful in distinguishing primary hematological diseases from secondary causes of pancytopenia and bicytopenia. However, vitamin B12 deficiency in developing countries is one of the most important causes of public health as well as in the etiology of pancytopenia and bicytopenia.

Clozapine-induced bicytopenia: An unusual side effect.

Agranulocytosis is a rare documented side effect of clozapine which can be associated with grave consequences. When it is associated with other blood dyscrasia, prognosis worsens further. In literature, there are very few cases of pancytopenia and bicytopenia caused by clozapine. We present a case of bicytopenia (reduced white and red blood cells' counts) caused by clozapine within a month of therapy and complicated by a Klebsiella pneumoniae infection. Patient improved in 3 weeks after stopping clozapine along with medical management in the Intensive Care Unit. Such side effects, though rare, can be life threatening and warrants intermittent complete blood monitoring besides regular assessment of granulocytes and neutrophils when any patient is prescribed clozapine.

Clinico-haematological profile of patients with bicytopenia.

Bicytopenia is the reduction of any of the two cell lines of blood, i.e., erythrocytes, leukocytes or platelets. Many studies are done on pancytopenia but very few studies exist in the literature evaluating the spectrum of aetiologies of bicytopenia. To date, no study is available on bicytopenia in adults. We aimed to study the clinico-haematological profile of patients with bicytopenia and to investigate the different aetiologies of bicytopenia. Four hundred patients with bicytopenia admitted to KLE Dr. Prabhakar Kore Charitable Hospital and Medical Research Centre were selected using systematic random sampling and included in the study. Their clinical profiles and haematological parameters were evaluated. Bicytopenia was observed in all ages with the mean age being 30.7 years. The occurrence of bicytopenia in different age groups was 6% in neonates, 7% in infants, 25% in children, 17% in teenagers, 85% in adults and 11% in elderly. The most common bicytopenia observed was anaemia with thrombocytopenia (61%) followed by anaemia with leukopenia (26%) and leukopenia with thrombocytopenia (13%). The male to female ratio was 1.6:1. The most common aetiology of bicytopenia was found to be non-malignant (56%) followed by infectious (31.7%), malignant (8.3%) and drug-induced (4%). Megaloblastic anaemia was the predominant aetiology among the non-malignant group, closely followed by immune thrombocytopenic purpura and alcoholic liver disease. The most common infectious disease was dengue (12%). Signs like lymphadenopathy, splenomegaly and hepatomegaly were most significantly associated with haematological malignancies (p < 0.001). Pallor, bleeding, hepatomegaly and splenomegaly were most frequent in non-malignant conditions (p < 0.001). Fever and lymphadenopathy were most frequent in the infectious category (p < 0.001). The most prevalent signs in drug-induced aetiology were lymphadenopathy, hepatomegaly and splenomegaly (p < 0.001). Bicytopenia is a good haematological indicator for many non-malignant and malignant diseases. Knowledge of its aetiologies can help in the diagnosis and efficient management of patients.

Hepatic cytolysis and bicytopenia after 15 days of four-drug tuberculosis treatment in Senegal.

We report the case of a 31-year-old immunocompetent woman residing in Senegal, with localized microscopy-proved pulmonary tuberculosis, complicated by macrophage activation syndrome and associated with viral hepatitis B, identified due to hepatic cytolysis and a bicytopenia.

Severe dengue due to secondary hemophagocytic lymphohistiocytosis: A case study.

Dengue, transmitted by the mosquito Aedes aegypti affects millions of people worldwide every year. Dengue induced hemophagocytic lymphohistiocytosis (HLH) is a serious condition and may prove fatal if not detected early and treated appropriately. Diagnosis of HLH is challenging and usually missed as clinical and laboratory findings are nonspecific. Moreover, the pathophysiology of the systemic inflammatory response syndrome and/or sepsis is remarkably similar to HLH. Secondary HLH following infection by the dengue virus is now being increasingly recognized as a cause of severe form of the disease. We report a case of dengue associated HLH in an otherwise healthy person who deteriorated during the course of hospitalization. A disproportionately high ferritin level and persistent bicytopenia prompted investigations for HLH. Diagnosis of dengue fever with virus-associated hemophagocytic syndrome was established according to the diagnostic criteria laid down by the Histiocyte Society. We discuss the diagnosis and management of this complex case and try to generate awareness about dengue induced HLH as one of the possible causes for severe manifestations of this infection.

[Evans syndrome in infants]. / Síndrome de Evans en lactantes.

BACKGROUND: Evans syndrome is characterized by the reduction of at least two blood cell lineages in the absence of other diagnoses; it was previously described as the simultaneous or sequential development of autoimmune hemolytic anemia and immune thrombocytopenia with unknown etiology. An incidence of 37% and mortality rate of 10% were reported for Evans syndrome. CLINICAL CASES: We report the clinical presentation and evolution of Evans syndrome in two infants who were initially diagnosed with immune thrombocytopenia. The clinical diagnosis was supported on complementary studies, where hematological disorders were corroborated. Both cases received treatment with steroids and intravenous immunoglobulin. CONCLUSIONS: For the management of children with thrombocytopenia, the pediatrician must analyze for other cell lineage disorders. In the cases that we report here, we found the presence of autoimmune hemolytic anemia and monocytosis. Therefore, infectious and immunological studies must be included. The first-line treatment of choice are steroids, and intravenous immunoglobulin can be considered if severe immune thrombocytopenia is associated, as observed in these cases.

Rectal Carcinoma Case Causing Bicytopenia.

Although conditions leading to bicytopenia and pancytopenia secondary to infiltrative diseases of the bone marrow are seen, a profound anemia or hemorrhages are frequently observed in such cases. As bone marrow infiltrations may be associated with primary hematological diseases such as leukemia, lymphoma or myeloma, rarely they may also be associated with solid tumor metastases. Here we have presented a case of rectal carcinoma causing profound bicytopenia dependent on diffuse bone marrow involvement.