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Focus: Blood Cancer JAN-MAR 17 www.singhealth.com.sg A SingHealth Newsletter for Medical Practitioners MCI (P) 027/11/2016 FOCUS: BLOOD CANCER Haematologic Emergencies An Overview of in the General Practice Myeloproliferative Neoplasms When to Suspect Approach to an Adult with Myeloma in Primary Care Lymphadenopathy in Primary Care SingHealth Duke-NUS Academic Medical Centre • Singapore General Hospital • KK Women’s and Children’s Hospital • Sengkang Health • National Cancer Centre Singapore • National Dental Centre of Singapore • National Heart Centre Singapore • National Neuroscience Institute • Singapore National Eye Centre • SingHealth Polyclinics • Bright Vision Hospital Medical Appointments: 6321 4402 (SGH) Focus: Update Blood Cancer 6436 8288 (NCCS) Haematologic Emergencies in the General Practice Adj Assoc Prof Wong Gee Chuan, Senior Consultant, Department of Haematology, Singapore General Hospital; SingHealth Duke-NUS Blood Cancer Centre Patients with malignant haematological diseases may present with dramatic and life-threaten- ing complications. General physicians must be able to recognise these conditions as prompt treatment can be life-saving. Hyperleukocytosis and leukostasis and febrile neutropaenia in patients with haematologic malignancies are two such conditions highlighted in this article. mental state and unsteadiness in gait. patient presents with a high white cell HYPERLEUKOCYTOSIS AND There is also increased risk of intracra- count and symptoms suggestive of tis- LEUKOSTASIS IN HAEMATOLOGIC nial haemorrhage. sue hypoxia. MALIGNANCIES Besides affecting the central nervous Hyperleukocytosis has been variably system, eyes and lungs, other manifes- Leukostasis constitutes a medi- defined as a total white cell count tations include myocardial ischaemia, cal emergency. Prompt treatment (WBC) of 50 x 109/L or 100 x 109/L. Leu- limb ischaemia or bowel infarction. is indicated. If left untreated, the kostasis is a medical emergency charac- Leukostasis may also occur where the one-week mortality can be as high terised by hyperleukocytosis and symp- clinical picture is less typical and at a as 40%. Symptomatic patients have toms of tissue hypoperfusion. It is most WBC lower than the arbitrarily defined a worse prognosis when compared commonly seen in patients with acute figures, especially in rapidly increasing to asymptomatic patients with hy- myeloid leukaemia (AML) or chronic blasts counts. perleukocytosis alone. myeloid leukaemia (CML) in blast crisis. INVESTIGATIONS The patient should be urgently Symptoms of leukostasis occur less Besides elevated WBC and blasts in referred to the Emergency De- frequently in patients with acute lym- the FBC, evidence of tumour lysis syn- partment of a hospital, where he phoblastic leukaemia (ALL) or chronic drome (TLS) may be present in up to should be rapidly stabilised. Rap- lymphocytic leukaemia (CLL) unless the 10% of patients with leukostasis. These id cytoreduction can be achieved WBC exceeds 150 x 109/L. include raised creatinine, hyperuricae- with chemotherapy or leukapher- mia, hyperkalaemia, hyperphosphate- esis. This should be accompanied Pathologically, it is characterised by in- mia and hypocalcaemia. Disseminated by tumour lysis syndrome prophy- travascular accumulation of blasts in the intravascular coagulation (DIC) may laxis and aggressive hydration and microvasculature, resulting in increased manifest with thrombocytopaenia and allopurinol. Specialised, supportive blood viscosity and decreased perfu- an abnormal coagulation profile. care such as mechanical ventila- sion. It is also postulated that local hy- tion for respiratory failure may be poxaemia may be exacerbated by the MANAGEMENT required. high metabolic activity of the dividing The diagnosis requires a high degree of blasts and release of cytokines. suspicion. It is made clinically when a SIGNS AND SYMPTOMS Clinically, this should be suspected when the full blood count (FBC) shows hyperleukocytosis in a patient present- ing with respiratory or neurologic dis- Hyperleukocytosis in tress. Patients can present with dys- peripheral blood film pnoea and hypoxia which can be picked of a chronic myeloid leukaemia patient. up on pulse oximetry. Neurologic signs and symptoms include visual chang- This image was originally published in ASH Image es, headache, dizziness, change in Bank. Peter Maslak. Hyper- leukocytosis – CML - 1. ASH Image Bank. 2010; image number-1022. © the Ameri- can Society of Hematology. 2 Jan-Mar 2017 • Unexplained fever – neutropae- nic fever with neither a clinical fo- cus of infection nor an identified pathogen RISK OF COMPLICATIONS The IDSA guideline considers low-risk patients as those who are expected to be neutropaenic (ANC <500 cells/ μL) for ≤7 days and those who have no active comorbidities or evidence of sig- nificant hepatic or renal dysfunction. High-risk patients are those who are ex- pected to be neutropaenic (ANC <500 cells/μL) for >7 days. Patients with neu- tropaenic fever who have ongoing co- morbidities or evidence of significant hepatic or renal dysfunction are also considered to be high-risk, regardless of the neutropaenia duration. Assessment of the risk of complications is crucial in patients with neutropaenic fever as this will dictate the approach to therapy, such as the need for inpatient admission and intravenous antibiotics. CT scan of patient with fungal pneumonia PATHOGENESIS This image was originally published in ASH Image Bank. Peter Maslak. Fungal pneumonia - 1. ASH Image Bank. 2007; image num- Chemotherapy-induced mucositis and ber-3475. © the American Society of Hematology. seeding of the bloodstream from en- dogenous flora in the gastrointestinal patients as a single oral temperature tract is believed to cause the majority FEBRILE NEUTROPAENIA IN of >38.3˚C (101˚F) or a temperature of of neutropaenic fevers. Immune defects PATIENTS WITH HAEMATOLOGIC >38.0˚C (100.4˚F) sustained for >1 hour. related to the underlying haematologic MALIGNANCIES malignancy and the immunosuppres- Neutropaenia is an absolute neutrophil sive effects of chemotherapy are other Febrile neutropaenia (FN) is one of the count (ANC) <1500 cells/μL, and severe contributory factors to the pathogen- most serious adverse events in patients neutropaenia is defined as ANC <500 esis of neutropaenic fever. with haematologic malignancies treated cells/μL or that is expected to decrease with chemotherapy. Since the magni- below 500 cells/μL during the next 48 An infectious source is identifiable in tude of the inflammatory response may hours, and profound neutropaenia is an approximately 20-30% of febrile neu- be muted in the neutropaenic patients, ANC <100 cells/μL. The risk of clinically tropaenic episodes. a fever may be the earliest and only important infections rises as the ANC sign of infection. Infections in these pa- decreases. About 80% of identified infections arise tients can progress rapidly, leading to from the patient’s endogenous flora. life-threatening complications. Initial neutropaenic fever syndromes Gram-positive bacteria (e.g. Staphy- can be classified into 3 categories: lococcus aureus, Enterococcus spp, Streptococcus pneumonia) are the FN is considered a medical emer- • Microbiologically documented in- most common causes of infections in gency. Prompt initiation of broad- fection – neutropaenic fever with the febrile neutropaenic patients. spectrum antibiotics is necessary a clinical focus of infection and an to avoid progression to sepsis associated pathogen However, infections caused by gram- and possibly death. negative bacteria (e.g. Pseudomonas • Clinically documented infection – aeruginosa, Klebsiella spp, Escherichia neutropaenic fever with a clinical coli) are associated with most serious DEFINITIONS focus (e.g. pneumonia) but with- consequences. The Infectious Diseases Society of Amer- out the isolation of an associated ica (IDSA) defines fever in neutropaenic pathogen 3 Medical Appointments: 6321 4402 (SGH) Focus: Update Blood Cancer 6436 8288 (NCCS) Invasive fungal infections are more common in high-risk patients with prolonged fever REFERENCES syndromes, with Candida and Aspergillus Hyperleukocytosis and Leukostasis in Haematologic Malignancies spp accounting for most of these infections. 1. Porcu P, Cripe LD, Ng EW et al. Hyperleukocytic leukemias and leukostasis: a re- Viral infections, including infections caused view of pathophysiology, clinical presentation and management. Leuk Lymphoma by respiratory viruses and human herpes 2000; 39:1 viruses, are also more common in high-risk 2. Lester TJ, Johnson JW, Cuttner J. Pulmonary leukostasis as the single worst prog- patients with neutropaenia. nostic factor in patients with acute myelocytic leukemia and hyperleukocytosis. Am J Med 1985; 79:43 MANAGEMENT It is critical to recognise neutropaenic fe- 3. Azoulay E, Fieux F, Moreau D et al. Acute monocytic leukemia presenting as acute respiratory failure. Am J Respir Crit Care Med 2003; 167:1329 ver early and for empiric broad-spectrum antibiotics to be initiated promptly to 4. Porcu P, Farag S, Marcucci G et al. Leukocytoreduction for acute leukemia. Ther avoid progression to a sepsis syndrome Apher 2002; 6:1 and possible demise. Febrile Neutropaenia in Patients with Haematologic Malignancies The managing oncologist can educate and 1. Freifeld AG, Bow EJ, Sepkowitz KA et al. Clinical practice guideline for the use of instruct the patient and their caregivers to antimicrobial agents in neutropenic patients with cancer: 2010 update by the Infec- recognise symptoms that require
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