Exchange transfusion in the management of critical pertussis in young infants: a case series.

BACKGROUND AND OBJECTIVES: It is proposed that severe leucocytosis mainly contributes to pulmonary hypertension by blocking pulmonary capillaries and restricting blood flow. Exchange transfusion (ET) in pertussis has been demonstrated as a safe and useful technique for depleting the leucocyte mass. We aim to discuss four cases of pertussis-induced respiratory distress and the effectiveness of ET in such a setting. MATERIALS AND METHODS: We conducted a retrospective case series at the Infectious Disease Department of Children's Hospital 2 in Ho Chi Minh City, Vietnam, and included four pertussis patients that were confirmed by PCR tests on respiratory secretions, presented with severe leucocytosis and respiratory distress and required mechanical ventilation. RESULTS: Among the included patients, three underwent a double volume ET for leucodepletion, two of whom were discharged after the procedure with proper vitals and laboratory test results. On the other hand, one patient died despite ET, performed late in the course of the disease. Exchange transfusion was not performed in the last patient who died as well. CONCLUSION: Early ET may be a useful and rapid life-saving treatment in children with critical pertussis and severe leucocytosis before cardiopulmonary complications appear.

Hyperleucocytosis in paediatric acute myeloid leukaemia - the challenge of white blood cell counts above 200 × 109 /l. The NOPHO experience 1984-2014.

Hyperleucocytosis in paediatric acute myeloid leukaemia (AML) is associated with increased morbidity and mortality. We studied hyperleucocytosis in 890 patients with AML aged 0-18 years registered in the Nordic Society of Paediatric Haematology and Oncology (NOPHO) registry, with special focus on very high white blood cell counts (WBC >200 × 10/l). Eighty-six patients (10%) had WBC 100-199 × 109 /l and 57 (6%) had WBC ≥200 × 109 /l. Patients with WBC ≥200 × 109 /l had a high frequency of t(9;11) and a paucity of trisomy 8. Due to the high frequency of deaths within the first 2 weeks (30% vs. 1% for all others), overall survival in this group was inferior to patients with WBC <200 × 109 /l (39% vs. 61%). Main cause of early death was intracranial haemorrhage and leucostasis. Twenty-six per cent of these patients never started antileukaemic protocol therapy. Leukapheresis or exchange transfusion was used in 24% of patients with hyperleucocytosis without impact on survival. Patients with hyperleucocytosis surviving the first week had identical survival as patients with lower WBC. We conclude that death within the first days after diagnosis is the major challenge in patients with high WBC and advocate rapid initiation of intensive chemotherapy.

A retrospective observational study of leucoreductive strategies to manage patients with acute myeloid leukaemia presenting with hyperleucocytosis.

Acute myeloid leukaemia (AML) patients with hyperleucocytosis have higher early mortality, lower complete remission (CR) and overall survival (OS). Whether different pre-induction leucoreduction strategies can improve outcome is unknown. A single centre retrospective cohort study was conducted on AML patients with a white blood cell count (WBC) >100 × 10(9) /l between 1987 and 1997, and on all AML patients between 1998 and 2006, to determine (a) the effect of four different leucoreductive strategies (leukapheresis, hydroxycarbamide, leukapheresis and hydroxycarbamide or no pre-induction leucoreduction) on early (day 28) mortality, CR, and OS; and (b) whether a high presenting WBC remains a negative predictor of OS in patients surviving induction (first 28 d). In the 1998-2006 cohort (n = 702), higher WBC was associated with higher early mortality and lower OS but its effects were greatly diminished in patients who survived the first 28 d (Hazard Ratio 1·094 vs. 1·002). A WBC of 34·1 × 10(9) /l had the highest sensitivity (75·6%) and specificity (67·4%) for early mortality. None of the four leucoreduction strategies differed significantly in early mortality, CR, or OS in patients with WBC>100 × 10(9) /l (n = 166). The number of leucostatic signs was a significant predictor of early mortality (P < 0·0001) and OS (P = 0·0007). The results suggest that AML patients with hyperleucocytosis should be induced, if eligible, without pre-induction leucoreduction.

Management of newly diagnosed chronic myeloid leukaemia during a twin pregnancy using leucapheresis: Case report and review of the literature.

A case of chronic myeloid leukaemia diagnosed as an incidental finding in a 32-year-old woman, pregnant with twins at 11 weeks gestation, is presented. Management of the patient was with leucapheresis and supportive care until spontaneous delivery of two morphologically normal infants (one male, one female) at 37 weeks gestation. Special considerations while employing leucapheresis in pregnant patients are discussed.

Hyperleucocytosis as an unusual presentation of hypereosinophilic pneumonitis with acute respiratory distress syndrome.

Idiopathic hypereosinophilic syndrome in children is a rare disease. Even with improved understanding of eosinophilic disorders and recent advances in diagnostic modalities, evaluation of hypereosinophilia remains challenging due to heterogeneous etiologic factors. In acute clinical settings, the management plan is often unclear as the condition is not encountered frequently. It is often associated with leucocytosis, but hyperleucocytosis causing multiorgan dysfunction in the absence of malignancy is rarely seen. A previously well 5-year-old boy presented to our emergency room with a 2-week history of fever, progressive cough and dyspnoea, rapidly progressing to respiratory failure and acute respiratory distress syndrome. Hyperleucocytosis with hypereosinophilia on peripheral blood film, bilateral pulmonary infiltrates on X-ray and ground glass opacities suggested hypereosinophilic syndrome with secondary acute respiratory distress syndrome. Owing to severe and rapidly increasing leucocytosis, malignancy was highly suspected, but it was ruled out along with secondary hypereosinophilic syndrome after extensive investigations, and acute respiratory distress syndrome in this child was attributed to Idiopathic Hypereosinophilic Syndrome. Eosinophilia had a dramatic response to high dose corticosteroid therapy. To conclude, in patients with hypereosinophilic syndrome, possibility of progression to acute respiratory distress syndrome should be anticipated and managed accordingly.

Hyperleukocytosis and leukostasis: management of a medical emergency.

INTRODUCTION: Hyperleukocytosis is defined as a white blood cell count greater than 100,000/mL in patients affected by acute leukemia and often it is associated with increased morbidity and mortality, that can be up to 40% if unrecognized. Areas covered: Risk factors include younger age, myelomonocytic or monocytic/monoblastic morphology, microgranular variant of acute promyelocitic leukemia and T-cell ALL, and some cytogenetic abnormalities. Poor prognosis due to high early death rate secondary to leukostasis. The mechanisms at the origin of leukostasis are still poorly understood. The management of acute hyperleukocytosis and leukostasis involves supportive measures and reducing the number of circulating leukemic blast cells, with careful monitoring of fluid balance, control of uric acid production and control of urine pH to prevent tumour lysis syndrome. Expert commentary: Several studies have been performed to ameliorate the outcome of this setting of patients. The high number of leukocytes may cause 3 main complications: disseminated intravascular coagulation (DIC), tumor lysis syndrome (TLS), and leukostasis. Although hyperleukocytosis and tumour lysis syndrome are still a challenge for clinicians, a better prognosis for these conditions is emerging in the last years.

Pertussis-like syndrome associated with adenovirus presenting with hyperleukocytosis: Case report.

Adenovirus is an infectious viral agent that causes variety of clinical presentations such as respiratory disease, conjunctivitis, and gastroenteritis. Hepatitis, pancreatitis, myocarditis, encephalitis, and disseminated infection are primarily seen in immunocompromised patients. Rarely, adenovirus infection can present with pertussis-like syndrome. Described here is case of pertussis-like syndrome associated with adenovirus presenting with hyperleukocytosis.

[Extreme leucocytosis can lead to an erroneous diagnosis of severe hypoxaemia. Description of a case]. / La leucocitosis extrema puede inducir a un diagnóstico erróneo de hipoxemia severa. Descripción de un caso.

The diagnosis and treatment of respiratory failure is a part of the anaesthesist's daily practice, as well as the hypoxaemia that is one of its physiological and analytical consequences. Patients with an extreme leucocytosis secondary to leukaemia can suffer an incorrect diagnosis of hypoxemia, called "pseudohypoxaemia". This is basically due to the rapid in vitro oxygen consumption, and is characterized by a low partial pressure of oxygen in arterial blood (PaO2) despite a normal oxygen saturation (SpO2) measured by pulse oximetry. Pseudohypoxaemia appears in patients with thrombocytosis or hyper-leucocytosis occurring during blastic crisis of a leukaemia. It must be suspected in patients with a discrepancy between the SpO2 measured by oximetry and the PaO2. In this context, pulse oximetry is the most accurate way to establish the diagnosis and to avoid unnecessary actions. We report the case of a patient with chronic myeloid leukaemia and extreme leucocytosis requiring emergency surgery, and diagnosed with pseudohypoxaemia during the perioperative period that led to a delay in the extubation of the patient.

Whole blood viscosity issues VII: The correlation with leucocytosis and implication on leukapheresis.

BACKGROUND: Blood hyperviscosity has been acknowledged to be a complicating factor in polycythaemia and hyperproteinaemia. Hyperleucocytosis has also been implicated in hyperviscosity and may be a basis for therapeutic leukapheresis. AIMS: This issue in the series seeks to determine the association and correlation between whole blood viscosity and white blood cell count (WBCC), with a view to advance the cause of a neglected clinical pathology index. MATERIALS #ENTITYSTARTX00026; METHODS: Based on archived clinical pathology data, 10,857 cases that were concomitantly tested for full blood count and total proteins in the 2008 calendar year were audited for hyperleucocytosis. Whole blood viscosity level was determined and compared in the group with leucocytosis relative to the group with leucopenia and normal WBCC. The confounding effects of age, gender and red blood cell indices were evaluated. The correlation between whole blood viscosity and WBCC was also determined. RESULTS: As a generalization, hypoviscosity is observed among individuals who presented hyperleucocytosis. There is no correlation (r = 0.20) between leucocytosis and blood viscosity. CONCLUSION: It is known that anaemia and thromboembolism, which can be associated with leucocytosis, predispose to hypoviscosity. The finding from this study provides evidence of association between hypoviscosity and leucocytosis. The absence of association and insignificant correlation between leucocytosis and hyperviscosity may be one explanation for ineffectiveness of therapeutic leukapheresis. Further, the non-correlation lends credence to specificity of blood viscosity, for which critical leucocytosis is not a substitute.