A rare complication of leukostasis with AML M4 patient: Microcirculatory dysfunction of upper extremities.

BACKGROUND: In children with acute myeloid leukemia, the incidence of hyperleukocytosis is 5-33%. Patients with AML and hyperleukocytosis have a higher early mortality rate than patients with nonhyperleukocytic AML because of the increased risk of severe pulmonary and neurologic complications. Leukapheresis provides rapid cytoreduction and reduces early mortality rates. CASE PRESENTATION: In this report, we present a case with microcirculatory failure of upper extremities as a rare symptom of hyperleukocytic AML M4 at initial presentation. CONCLUSIONS: Early diagnosis and treatment of patients with AML admitted to emergency services with these symptoms is too important to prevent from loss of extremities. Most of the complications of hyperleukocytosis can be reversible with early treatment.

Cardiac failure in a child with tuberculous meningitis as a complication of Paroxysmal sympathetic hyperactivity.

BACKGROUND: Paroxysmal sympathetic hyperactivity (PSH) is a disorder due to the loss of regulation of autonomic activity. The most common condition predisposing to the development of PSH is traumatic brain injury (TBI), followed by anoxic brain injury, stroke, tumors, and infections. Awareness about the condition and early recognition is important to avoid life threatening complications. CASE: We report a 4-year-old child with tuberculous meningitis with symptoms of PSH who developed cardiac failure. PSH episodes were treated with beta blocker, benzodiazepine, morphine, dexmedetomidine, baclofen, and tizanidine. Three weeks after readmission PSH episodes decreased and the patient was transferred to the general ward. CONCLUSIONS: PSH assessment tool has benefits such as monitoring the patient, evaluating response to treatment and early diagnosing PSH patients.

A multicentered study on efficiency of noninvasive ventilation procedures (SAFE-NIV)

Background/aim: To characterize the clinical course of noninvasive positive pressure ventilation (NIPPV) and high flow humidified nasal cannula ventilation (HFNC) procedures; perform risk analysis for ventilation failure. Material and methods: This prospective, multi-centered, observational study was conducted in 352 PICU admissions (1 month-18 years) between 2016 and 2017. SPSS-22 was used to assess clinical data, define thresholds for ventilation parameters and perform risk analysis. Results: Patient age, onset of disease, previous intubation and hypoxia influenced the choice of therapy mode: NIPPV was preferred in older children (p = 0.002) with longer intubation (p < 0.001), ARDS (p = 0.001), lower respiratory tract infections (p < 0.001), chronic respiratory disease, (p = 0.005), malignancy (p = 0.048) and immune deficiency (p = 0.026). The failure rate was 13.4%. sepsis, ARDS, prolonged intubation, and use of nasal masks were associated with NIV failure (p = 0.001, p < 0.001, p < 0.001, p = 0.025). The call of intubation or re-intubation was given due to respiratory failure in twenty-seven (57.5%), hemodynamic instability in eight (17%), bulbar dysfunction or aspiration in 5 (10.6%), neurological deterioration in 4 (8.5%) and developing ARDS in 3 (6.4%) children. A reduction of less than 10% in the respiration within an hour increased the odds of failure by 9.841 times (OR: 9.841, 95% CI: 2.0021­48.3742). FiO2 > 55% at 6th hours and PRISM-3 >8 were other failure predictors. Of the 9.9% complication rate, the most common complication was pressure ulcerations (4.8%) and mainly observed when using full-face masks (p = 0.047). Fifteen (4.3%) patients died of miscellaneous causes. Tracheostomy cannulation was performed on 16 children due to prolonged mechanical ventilation (8% in NIPPV, 2.6% in HFNC) Conclusion: Absence of reduction in the respiration rate within an hour, FiO2 requirement >55% at 6th hours and PRISM-3 score >8 predict NIV failure.

The evaluation of safety and efficacy of colistin use in pediatric intensive care unit: Results from two reference hospitals and review of literature.

Colistin, an old cationic polypeptide antibiotic, have been reused due to rising incidence of infections caused by multi-drug resistant (MDR) Gram-negative microorganisms and the lack of new antibiotics. Therefore, we evaluated safety and efficacy of colistin in treatment of these infections. This study included 104 critically ill children with a median age of 55,9 months between January 2011 and January 2016. Nephrotoxicity occurred in 11 (10.5%) patients. Nephrotoxicity occurred between the third and seventh day of treatment in 63% of colistin induced nephrotoxicity episodes. The subgroup analysis between the patients who developed nephrotoxicity during colistin treatment and those that did not, showed no significant difference in terms of age, underlying disease, cause for PICU admission and type of infection required colistin treatment, P values were 0.615, 0.762, 0.621, 0.803, respectively. All patients were receiving a concomitant nephrotoxic agent (P = 0,355). The majority of the patients (52%) were having primary or secondary immune deficiency in treatment failure group and the most common cause of PICU admission was sepsis in treatment failure group, P values were 0.007 and 0.045, respectively. Mortality attributed to colistin failure and crude mortality were 14.4% and 29.8%, respectively. In conclusion, colistin may have a role in the treatment of infections caused by multidrug-resistant Gram-negative bacteria in critically ill children. However, the patients have to be followed for side effects throughout colistin treatment, not for only early stage. And the clinicians should be aware of increase in the rate of nephrotoxicity in patients those have been receiving a concomitant nephrotoxic agent.

A fatal case of tuberculous meningitis in a child with juvenile idiopathic arthritis: a diagnostic challenge.

The prognosis of tuberculous meningitis, a rare form of extrapulmonary tuberculosis, depends on the stage of treatment initiation. We report a fatal case of tuberculous meningitis. The patient had received successive tumor necrosis factor (TNF) antagonists and abatacept to treat juvenile idiopathic arthritis, with negative results for polymerase chain reaction and acid-fast bacilli on smear, had normal cerebrospinal fluid (CSF) adenosine deaminase and glucose levels. Six weeks post-admission, the CSF culture demonstrated Mycobacterium tuberculosis. The altered immunological responses caused by anti-TNF treatment made the diagnosis challenging. Clinicians should bear this in mind and, if suspected, treatment should be initiated immediately.

A fatal case of tuberculous meningitis in a child with juvenile idiopathic arthritis: a diagnostic challenge

Abstract The prognosis of tuberculous meningitis, a rare form of extrapulmonary tuberculosis, depends on the stage of treatment initiation. We report a fatal case of tuberculous meningitis. The patient had received successive tumor necrosis factor (TNF) antagonists and abatacept to treat juvenile idiopathic arthritis, with negative results for polymerase chain reaction and acid-fast bacilli on smear, had normal cerebrospinal fluid (CSF) adenosine deaminase and glucose levels. Six weeks post-admission, the CSF culture demonstrated Mycobacterium tuberculosis. The altered immunological responses caused by anti-TNF treatment made the diagnosis challenging. Clinicians should bear this in mind and, if suspected, treatment should be initiated immediately.

Difficult diagnosis of invasive fungal infection predominantly involving the lower gastrointestinal tract in acute lymphoblastic leukaemia.

Invasive fungal infections are most commonly seen in immunocompromised patients and usually affect the respiratory system. Gastrointestinal system involvement of mucormycosis and invasive aspergillosis is rarely reported in childhood. Here we describe a 5 year old boy with acute lymphoblastic leukaemia who developed invasive fungal infection particularly affecting the lower gastrointestinal system to emphasise the difficulties in diagnosis and management of invasive fungal infections in immunocompromised patients.

Rare severe mycotic infections in children receiving empirical caspofungin treatment for febrile neutropenia

ABSTRACTEmpirical antifungal therapy is most often given to patients with leukemia. However breakthrough fungal infections under antifungal therapy are not uncommon. Four children, with hematologic malignant disease developed mycotic breakthrough infections while on empirical caspofungin treatment for a median of 14 (range 11-19) days. Trichosporon asahii was detected in the blood culture of two patients and Geotrichum capitatum in the other two (one patient also had positive cerebrospinal fluid culture). Because the patients' clinical situation worsened, voriconazole was empirically added for two patients three and five days before the agent was detected. The first sterile blood culture was obtained 3-7 days of voriconazole treatment. All patients reached clear cultures but one patient died. One patient with central nervous system infection with G. capitatum had severe neurological sequelae. Very severe fungal infections can occur during empirical caspofungin therapy. Therefore, patients should be followed closely.

Rare severe mycotic infections in children receiving empirical caspofungin treatment for febrile neutropenia.

Empirical antifungal therapy is most often given to patients with leukemia. However breakthrough fungal infections under antifungal therapy are not uncommon. Four children, with hematologic malignant disease developed mycotic breakthrough infections while on empirical caspofungin treatment for a median of 14 (range 11-19) days. Trichosporon asahii was detected in the blood culture of two patients and Geotrichum capitatum in the other two (one patient also had positive cerebrospinal fluid culture). Because the patients' clinical situation worsened, voriconazole was empirically added for two patients three and five days before the agent was detected. The first sterile blood culture was obtained 3-7 days of voriconazole treatment. All patients reached clear cultures but one patient died. One patient with central nervous system infection with G. capitatum had severe neurological sequelae. Very severe fungal infections can occur during empirical caspofungin therapy. Therefore, patients should be followed closely.

Comparison of endotracheal aspirate and non-bronchoscopic bronchoalveolar lavage in the diagnosis of ventilator-associated pneumonia in a pediatric intensive care unit.

Ventilator-associated pneumonia (VAP) is defined as pneumonia occuring in any period of mechanical ventilation. There is no optimal diagnostic method in current use and in this study we aimed to compare two non-invasive diagnostic methods used in diagnosis of VAP in children. This prospective study was conducted in 8 bedded Pediatric Intensive Care Unit at Ege University Children´s Hospital. Endotracheal aspiration (ETA) and non-bronchoscopic bronchoalveolar lavage (BAL) were performed in case of developing VIP after 48 hours of ventilation. Quantitative cultures were examined in Ege University Department of Diagnostic Microbiology, Bacteriology Laboratory. Fourty-one patients were enrolled in the study. The mean age of study subjects was 47.2±53.6 months. A total of 28 in 82 specimens taken with both methods were negative/negative; 28 had positive result with ETA and a negative result with non-bronchoscopic BAL and both results were negative in 26 specimens. There were no patients whose respiratory specimen culture was negative with ETA and positive with non-bronchoscopic BAL. These results imply that there is a significant difference between two diagnostic methods (p < 0.001). Negative non-bronchoscopic BAL results are recognized as absence of VAP; therefore, ETA results were compared with this method. ETA's sensitivity, specificity, negative and positive predictive values were 100%, 50%, 100% and 48% respectively. The study revealed the ease of usability and the sensitivity of non-bronchoscopic BAL, in comparison with ETA.

Trichosporon asahii, sepsis, and secondary hemophagocytic lymphohistiocytosis in children with hematologic malignancy.

Trichosporon asahii (T. asahii) is an uncommon fungal pathogen rarely seen in patients with hematologic malignancies. Although appropriate therapy is started, infection with T. asahii usually leads to mortality. Here, we describe two patients developed severe T. asahii infection and secondary HLH. Despite rapid identification of T. asahii and negative blood cultures achieved by prompt initiation of treatment with voriconazole, fever and pancytopenia, persisted and both developed hepatosplenomegaly, and their clinical state worsened. Bone marrow aspiraton revealed hemophagocytosis. Elevated ferritin, triglyceride levels were seen. The first patient did not receive HLH directed therapy and died with multiple organ dysfunctions. Prompt diagnosis and treatment of secondary HLH led to rapid improvement in clinical and laboratory abnormalities in the second patient and kept her alive. We suggest that HLH may present as a secondary condition, accompanying a severe infection with T. asahii may, at least in part, contribute to high mortality rates in these cases.

Leukapheresis in childhood acute leukemias: single-center experience.

Hyperleukocytosis in patients with leukemia is associated with early mortality, especially due to the pulmonary and neurological complications of leukostasis. The prompt use of leukapheresis may improve patients' survival in the initial treatment period. The medical records of all previously untreated acute leukemia patients were reviewed to determine whether there was hyperleukocytosis at presentation. This study summarizes a single-center experience of leukapheresis that was applied to 12 children with acute leukemia and hyperleukocytosis. The median leukocyte count at diagnosis was 589,000/mm(3) (range: 389,000-942,000/mm(3)) for ALL patients and 232,000/mm(3) (range: 200,000-282,000/mm(3)) for AML patients. A central venous catheter (CVC) was inserted, and leukapheresis procedures were repeated at 12-hour intervals. A total of 29 leukapheresis cycles were performed on 12 children. The median number of cycles of leukapheresis required by each patient was two (range: 1-4). The median absolute and percentage reductions in white blood cell (WBC) count after the first cycle of leukapheresis were 113,000/mm(3) (range: 55,000-442,000/mm(3)) and 36% (range: 16-57.4%), respectively. As a laboratory finding, mild hypocalcemia was the most frequently observed complication. No patients developed any other problem related to the procedure. Our results showed that leukapheresis is a safe and effective procedure if performed by experienced staff.

[Can increased intra-abdominal pressure (IAP) be treated more effectively with intravesical pressure measurement in high-risk patients?]. / Kariniçi basinç artisina yol açabilen hastaliklarin tedavisi intravezikal basinç ölçümü kontrolü altinda daha güvenle yapilabilir mi?

BACKGROUND: Increased intra-abdominal pressure (IAP) can result in multiorgan failure. Trauma, mechanical bowel obstruction (MBO), abdominal wall defects (AWD) and fluid resuscitation in septic shock are conditions associated with a high risk of increased IAP in children. It may be possible to treat these conditions more effectively using intravesical pressure measurement. METHODS: This prospective study was performed between December 2009 and October 2010. Intravesical pressure (IVP) measurement was used to determine IAP. The presence of Intra-abdominal hypertension (IAP >12 mmHg) and abdominal compartment syndrome (IAP >15 mmHg + new organ dysfunction) was evaluated in all clinical decisions. RESULTS: IVP monitoring was applied in all patients with abdominal trauma (14), AWD (8), fluid resuscitation for septic shock (7), and MBO (6). A diagnosis of ACS was determined in 14% of trauma patients and required surgery. IAH/ACS occurred in 38% of AWD cases, which required closure. IAH occurred in 43% of patients undergoing fluid resuscitation for septic shock, requiring decompressive intervention. IAH/ACS occurred in 50 % of MBO patients, all of whom required surgery. DISCUSSION: IVP measurement is a useful method to identify candidates for surgical treatment in cases of trauma and MBO. Similarly, IVP monitoring can facilitate the closure of abdominal wall defects and the application of fluid resuscitation in septic shock.

Hyperferritinemia in the critically ill child with secondary hemophagocytic lymphohistiocytosis/sepsis/multiple organ dysfunction syndrome/macrophage activation syndrome: what is the treatment?

INTRODUCTION: Hyperferritinemia is associated with increased mortality in pediatric sepsis, multiple organ dysfunction syndrome (MODS), and critical illness. The International Histiocyte Society has recommended that children with hyperferritinemia and secondary hemophagocytic lymphohistiocytosis (HLH) or macrophage activation syndrome (MAS) should be treated with the same immunosuppressant/cytotoxic therapies used to treat primary HLH. We hypothesized that patients with hyperferritinemia associated secondary HLH/sepsis/MODS/MAS can be successfully treated with a less immunosuppressant approach than is recommended for primary HLH. METHODS: We conducted a multi-center cohort study of children in Turkish Pediatric Intensive Care units with hyperferritinemia associated secondary HLH/sepsis/MODS/MAS treated with less immunosuppression (plasma exchange and intravenous immunoglobulin or methyl prednisolone) or with the primary HLH protocol (plasma exchange and dexamethasone or cyclosporine A and/or etoposide). The primary outcome assessed was hospital survival. RESULTS: Twenty-three children with hyperferritinemia and secondary HLH/sepsis/MODS/MAS were enrolled (median ferritin = 6341 µg/dL, median number of organ failures = 5). Univariate and multivariate analyses demonstrated that use of plasma exchange and methyl prednisolone or intravenous immunoglobulin (n = 17, survival 100%) was associated with improved survival compared to plasma exchange and dexamethasone and/or cyclosporine and/or etoposide (n = 6, survival 50%) (P = 0.002). CONCLUSIONS: Children with hyperferritinemia and secondary HLH/sepsis/MODS/MAS can be successfully treated with plasma exchange, intravenous immunoglobulin, and methylprednisone. Randomized trials are required to evaluate if the HLH-94 protocol is helpful or harmful compared to this less immune suppressive and cytotoxic approach in this specific population.

Soft tissue infection caused by Burkholderia cepacia in a child with polyarteritis nodosa.

Burkholderia cepacia belongs to a family of Burkholderia species previously described as Pseudomonas cepacia, especially in patients suffering from cystic fibrosis. There are also many studies about this agent in the last decade due to their life-threatening infections and ability to invade mucosal and cellular surfaces. Here, we report a case of soft tissue infection caused by B. cepacia in a child with an underlying condition of polyarteritis nodosa. Her complaints started at two months of age and she was on cyclosporine therapy. She was treated several times because of soft tissue infections especially in her extremities. The most common causative agents were Pseudomonas spp. and Escherichia coli, but recently, another soft tissue infection accompanied by fever and signs of sepsis had developed. All blood, urine and tissue (debrided from the necrotic area) specimens were incubated. Empirical antibiotherapy with clindamycin was started and cyclosporine therapy was discontinued. B. cepacia was grown in the tissue specimen culture and was only susceptible to carbapenems. Meropenem therapy was administered throughout 14 days with a daily dosage of 60 mg/kg, and she was treated successfully at least in this attack of soft tissue infection, which caused more severe sepsis and tissue damage than the previous infections with other agents.

Imatinib treatment alone in Philadelphia-positive acute lymphoblastic leukemia: is it enough?

BCR-ABL fusion gene t(9;22)(q34;q11) occurs in only 3% of pediatric acute lymphoblastic leukemia (ALL) cases. Previously, less than 40% of Philadelphia-positive ALL patients were cured with intensive chemotherapy. The use of imatinib (340 mg/m(2)/day) added to an intensive chemotherapy regimen has improved the outcome in this population at 3 years to an event-free survival of 80%. Imatinib treatment alone was administered after remission induction chemotherapy to a patient with Philadelphia-positive ALL who presented with serious chemotherapy toxicity, so that intensive chemotherapy could not be maintained. This is the only patient in the literature who survived remission for more than 2.5 years with imatinib treatment only.

A rescue therapy with a combination of caspofungin and liposomal amphotericin B or voriconazole in children with haematological malignancy and refractory invasive fungal infections.

Combination treatment of paediatric invasive fungal infections (IFIs) has rarely been reported. A total of 17 children with 19 IFI episodes were enrolled in the study. The median age of the patients was 5.3 (range 0.5-17) years. IFI was classified as proven in 4, probable in 12 and possible in 3 episodes. These patients received empiric antifungal treatment, which consisted of liposomal amphotericin B (LAmB) monotherapy for a median duration of 12 days (range 3-69 days). All patients were refractory to LAmB; therefore, caspofungin was added to the therapy in 11 patients. In the remaining six patients, LAmB was ceased and a combination of caspofungin and voriconazole was started. Among the patients who received caspofungin + LAmB, four did not show favourable response and the combination was switched to caspofungin + voriconazole. The median (range) and total duration of the therapy were 7 (3-14) days and 91 patient days for LAmB + caspofungin combination and 49 (7-126) days and 516 patient days for caspofungin + voriconazole combination. We found a favourable response rate of 68.4% in 16 proven or probable IFI episodes. Twelve-week survival rate of these patients was 75%. No serious side effect was observed among the patients. Our data suggest that combination antifungal therapy is safe and effective in children with haematological malignancies.

An unusual cause of multiple organ dysfunction syndrome in the pediatric intensive care unit: hemophagocytic lymphohistiocytosis.

OBJECTIVE: To report our experience in children with primary or secondary hemophagocytic lymphohistiocytosis (HLH) presented with multiple organ dysfunction syndrome (MODS) in pediatric intensive care unit (PICU). DESIGN: The records of patients with a diagnosis of HLH and MODS between January 2005 and January 2008 were reviewed. The patients' characteristics, treatment modalities, and outcomes were assessed. SETTING: PICU of Ege University Hospital. PATIENTS/SUBJECTS: Twelve children who were hospitalized in the PICU met the diagnostic criteria for HLH, and presented with MODS were entered into the study. RESULTS: The median age of the patients was 3 years (range, 2 months-15.5 years). Six patients had a history of parental consanguinity and two had an affected sibling. Five of the patients were classified as primary HLH. All of the patients had hepatosplenomegaly, elevated ferritin levels, hypofibrinogenemia, anemia, thrombocytopenia, and hemophagocytosis in bone marrow examination at presentation. The median Pediatric Logistic Organ Dysfunction score of the patients at onset was 51 (range, 12-62). Four patients had six, four had five, two had four, and the remaining two had three organ dysfunctions. Organ dysfunction, other than hematologic dysfunction which was present in all patients, was most commonly seen in hepatic (n = 11, 91.7%), respiratory (n = 11, 91.7%), and cardiovascular systems (n = 10, 83.3%). Although nine patients showed neurologic dysfunction including convulsion and coma, renal failure was detected in five patients. Eleven patients were supported with mechanical ventilation and four patients required hemodialysis. Eight patients were treated according to the HLH 2004 treatment protocol, consisting of cyclosporine A, etoposide, and dexamethasone. The remaining four patients received only intravenous immunoglobulin and supportive treatment. Seven of the patients died. CONCLUSION: HLH is a frequently lethal disease and with a clinical presentation similar to severe sepsis, MODS, disseminated intravascular coagulation, or septic shock, which are frequent diagnoses in the PICU. In the PICU, HLH should be considered in the case of prolonged fever, splenomegaly, cytopenia, and MODS. It is important for pediatricians and particularly pediatric intensivists to know the diagnostic criteria and possible clinical presentations of HLH so treatment is initiated promptly.

Single-center experience: use of recombinant factor VIIa for acute life-threatening bleeding in children without congenital hemorrhagic disorder.

Coagulopathy is an important cause of mortality in critically ill children. Traditional therapies to correct coagulopathy lead to great time delays and cause fluid overload in patients. The authors report the effectiveness and safety of the activated recombinant factor VII (rFVIIa) administration in a series of 13 nonhemophiliac children with acute, life-threatening bleeding. In this retrospective study, the records of the patients who were not diagnosed with congenital hemorrhagic disorder and were administered rFVIIa due to any other reason in Ege University Faculty of Medicine, Department of Pediatrics, between February 2002 and February 2007 were reviewed retrospectively. Thirteen nonhemophiliac patients with acute life-threatening bleeding and ages ranging from 2 days to 15 years received rFVIIa over a 5-year period. Three patients were diagnosed with hemaphagocytic lymphohistiocytosis, 4 with prematurity, sepsis, and disseminated intravascular coagulation (DIC), 5 with sepsis, multiple organ dysfunction syndrome, and DIC, and 1 with acute liver failure. Severe bleeding resulted from pulmonary (n = 3), lower gastrointestinal system (n = 2), esophagus varices (n = 1), pulmonary and gastrointestinal system (n = 4), pulmonary, gastrointestinal system, and intracranial hemorrhage (n = 1), and gastrointestinal system and intracranial hemorrhage (n = 2). Median frequency of rFVIIa administration was 3 per patient (range 2-15) and median dose of rFVIIa was 90 microg/kg, ranging from 60 to 135 microg/kg each administration. All of the patients were given fresh frozen plasma and if necessary platelet transfusion (n = 10) or fibrinogen concentrate (n = 3) before administration of rFVIIa. In 6 patients, lack of success to control bleeding by conventional methods was the only cause to start rFVIIa. In 7 patients, the need for volume restriction was also a significant contributing factor in deciding to start rFVIIa. Median PT was 32.9 s (range: 19-65) before rFVIIa administration and it was decreased to 11.6 s (range: 10.7-12.8), 2-3 h after rFVIIa infusion. Bleeding was stopped completely in 10 patients at least for 24 h and decreased in 3 patients 30-45 min after rFVIIa administration. Two patients had thrombotic complications attributed to rFVIIa administration. No other complication was observed in the other patients. In this retrospective study, rFVIIa was found to be effective at controlling severe hemorrhagic symptoms of different etiologies in children without congenital hemorrhagic disorder. In addition to the rapid control of bleeding, administration of this agent improved fluid balance and led to a reduction in blood product requirements in critically ill children. However, survival was still poor (23%), and 2/13 (15.4%) patients developed venous and arterial thrombosis within 3 h of treatment. The authors emphasize that in acquired, acute life-threatening bleeding, simultaneous administration of rFVIIa with conventional treatment may contribute to patient survival. However, the risk of thromboembolism should be considered before this treatment is given.

Intense myelofibrosis in a child: unusual result of EBV-associated hemophagocytic lymphohistiocytosis.

A previously healthy 12-year-old girl was admitted to the intensive care unit with severe pulmonary bleeding. Her history revealed that she had suffered from high fever, fatigue, sore throat, myalgia and generalized rash for two weeks. Physical examination revealed hepatosplenomegaly. Laboratory investigation showed pancytopenia associated with unusual high levels of serum ferritin, triglyceride and lactate dehydrogenase (LDH) and low fibrinogen levels. Apparent hemophagocytosis was seen in bone marrow aspiration. Bone marrow biopsy revealed myelofibrosis, and confirmed hemophagocytosis. IgM for Epstein-Barr virus (EBV) viral capsid antigen was found to be positive. She received chemotherapy for 10 days according to hemophagocytic lymphohistiocytosis (HLH)-2004 treatment protocol, since the symptoms persisted despite supportive therapy and intravenous immunoglobulin (IVIG) administration. However, the clinical status and laboratory findings did not respond to treatment and she died from severe pulmonary bleeding associated with prolonged ventilator support and sepsis. Intense myelofibrosis, which is reported rarely, particularly in patients with EBV-related HLH, contributed to this fatal prognosis.