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CHAPTER 92: Acute Leukemia  865


                    complication is catheter-related blood stream infections and sepsis; one   CHAPTER  Acute Leukemia
                    series observed systemic infections in up to 12% of patients undergoing
                    plasma exchange for TTP and HUS.  Although routine surveillance   Karen-Sue B. Carlson
                                               221
                    cultures are not recommended, the critical care physician should have a  92
                    high suspicion of systemic infection in this population with indwelling
                    central venous catheters as occult infections have been described in this
                    population.  Lastly, catheter-related thrombosis or obstruction has been
                            222
                    reported in this series as a complication of plasma treatment.  The risks   KEY POINTS
                                                               221
                    associated with plasma transfusion including allergic reaction, TRALI
                    and citrate toxicity are among the complications of plasma exchange     • Untreated acute leukemia is rapidly fatal, but a percentage of
                    for TTP and HUS. Citrate toxicity, causing hypocalcemia and metabolic   patients can be cured. In the absence of cure, many can achieve a
                    alkalosis, can be avoided by routine administration of calcium gluconate   significant duration of high-quality life depending on preleukemia
                    during plasma exchange therapy. Serial monitoring of ionized calcium   comorbidities, and should therefore be considered for therapy.
                    levels is also recommended. The diagnosis of TRALI should be suspected     • Medical complications of the acute leukemias are often reversed
                    in patients undergoing therapeutic plasma exchange who develop respi-  with treatment of the underlying disease.
                    ratory deterioration. 223-225  Cessation of plasma infusion and blood bank     • Bleeding or infectious complications account for the majority of
                    workup such as antibody testing should be performed in suspected cases.
                                                                            deaths in patients with acute leukemias.
                                                                              • Laboratory findings define and are prominent in tumor lysis syn-
                                                                            drome and include hyperkalemia, hyperphosphatemia, hyperuri-
                     KEY REFERENCES                                         cemia, and hypocalcemia.
                                                                              • Rasburicase can be used to treat hyperuricemia, but must be
                        • Caprioli J, Noris M, Brioschi S, et al. Genetics of HUS: the impact   avoided for patients with known hypersensitivity, methemoglobin-
                       of MCP, CFH, and IF mutations on clinical presentation, response   emia, or G6PD deficiency.
                       to treatment, and outcome. Blood. 2006;108(4):1267-1279.
                                                                              • Urinary alkalinization is not recommended for prevention of
                        • Fujimura Y, Matsumoto M. Registry of 919 patients with throm-  tumor lysis syndrome.
                       botic microangiopathies across Japan: database of Nara Medical
                       University during 1998-2008. Intern Med. 2010;49(1):7-15.    • Cytogenetics and molecular studies are important prognostic indi-
                                                                            cators in AML and ALL.
                        • George JN. How I treat patients with thrombotic thrombocytope-
                       nic purpura: 2010. Blood. 2010;116(20):4060-4069.      • Prophylactic platelet transfusion is indicated for thrombocytope-
                                                                            nia  associated with acute leukemia.
                        • Levy GG, Nichols WC, Lian EC, et al. Mutations in a member of     • Disseminated intravascular coagulation (DIC) should be aggres-
                       the  ADAMTS  gene  family  cause  thrombotic  thrombocytopenic   sively treated with transfusion support with fresh frozen plasma
                       purpura. Nature. 2001;413(6855):488-494.             and cryoprecipitate during the initial treatment of acute leukemias.
                        • Moake JL, Rudy CK, Troll JH, et al. Unusually large plasma     • Acute promyelocytic leukemia (APL) should be suspected in
                       factor VIII: von Willebrand factor multimers in chronic relaps-  patients with pancytopenia and severe DIC.
                       ing thrombotic thrombocytopenic purpura.  N Engl J Med.
                       1982;307(23):1432-1435.                               • All-trans retinoic acid (ATRA) therapy should be rapidly initiated if
                        • Noris M, Mescia F, Remuzzi G. STEC-HUS, atypical HUS and   APL is suspected, and invasive procedures including placement of a
                                                                            central venous catheter should be avoided until the DIC has resolved.
                       TTP are all diseases of complement activation. Nat Rev Nephrol.
                       2012;8(11):622-633.                                    • Rapid cytoreduction by hydroxyurea and leukapheresis are main-
                                                                            stays of therapy for hyperleukocytosis.
                        • Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving
                       protease in acute thrombotic thrombocytopenic purpura. N Engl J
                       Med. 1998;339(22):1585-1594.                       INTRODUCTION
                        • Würzner R, Riedl M, Rosales A, Orth-Höller D. Treatment of
                       enterohemorrhagic Escherichia coli-induced hemolytic uremic   Acute  leukemias  are  a  collection  of  bone  marrow  and  lymphoid  dis-
                       syndrome (eHUS).  Semin Thromb Hemost. 2014; Epub ahead   orders that result from establishment of a malignant stem cell popula-
                       PMID 24802085.                                     tion. Presentation of a patient to medical care with a suspected acute
                        • Zheng XL, Chung D, Takayama T, Majerus E, Sadler J,     leukemia should be considered a medical emergency, and rapidly
                                                                          involve a specialist in hematologic malignancies and referral to a tertiary
                       Fujikawa K. Structure of von Willebrand factor-cleaving pro-
                       tease (ADAMTS13), a metalloprotease involved in thrombotic     care facility with expertise in treatment of patients with acute leukemia.
                                                                          Left untreated, acute leukemias are rapidly fatal within days to weeks of
                       thrombocytopenic purpura.  J Biol Chem. 2001;276(44):
                       41059-41063.                                       presentation, but with appropriate supportive measures and therapeutic
                                                                          interventions a significant number of patients are cured. For those in
                        • Zheng XL, Sadler JE. Pathogenesis of thrombotic microangiopa-  whom a cure cannot be achieved, there is still the potential for a sub-
                       thies. Annu Rev Path Mech Dis. 2008;3:249-277.     stantial period of good quality life. Because of the acuity of these diseases
                        • Zipfel PF, Neumann HP, Jozsi M. Genetic screening in haemolytic   and consequences of their treatment, it is not infrequent that patients
                       uraemic syndrome.  Curr Opin Nephrol Hypertens. 2003;12(6):   with acute leukemias are seen in the setting of a medical ICU.  It is espe-
                                                                                                                     45
                       653-657.                                           cially important to understand that acute leukemias develop rapidly and
                                                                          the disease itself profoundly compromises an individual’s performance
                                                                          status and comorbidities; however, with rapid and appropriate therapy,
                                                                          these  leukemia-induced  complications  are  often  reversed.  In  order  to
                    REFERENCES                                            appropriately target  care  from  the  perspective  of  the  intensivist,  it  is
                                                                          important to understand the disease pathophysiology, overall prognostic
                    Complete references available online at www.mhprofessional.com/hall  evaluation, and complications unique to leukemia treatment regimens.









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