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Hematological emergencies 2

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continuation of hematological emergencies

Publicada em: Saúde e medicina
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Hematological emergencies 2

  1. 1. HEMATOLOGICAL EMERGENCIES-2 Ajay Kumar Yadav PGY3,Medicine IOM-TUTH, Kathmandu
  2. 2. LAYOUT • Hyperleucocytosis and leucostasis • Spinal cord compression • Human Antibody Infusion Reactions • Neutropenic enterocolitis
  4. 4. INTRODUCTION • Hyperleukocytosis : Refers to lab abnormality that has been variably defined as a total leukemia blood cell count greater than 50,000/microL or 100,000/microL. • Leukostasis : Aka Symptomatic Hyperleukocytosis  Medical emergency  Most commonly seen in patients with AML or CML in blast crisis.  Characterized by an extremely elevated blast cell count and symptoms of decreased tissue perfusion.
  5. 5. Cont.. • Leucostasis is a Pathologic diagnosis  white cell plugs are seen in the microvasculature. • Clinically diagnosed empirically when a patient with leukemia and hyperleukocytosis presents with respiratory or neurological distress. • Prompt t/t is indicated since, if left untreated, the one-week mortality rate is approx. 20 to 40 pc. • The frequency of hyperleukocytosis is 5–13% in AML and 10–30% in ALL.
  6. 6. EPIDEMIOLOGY • AML: • More common with myelomonocytic (FAB-M4) leukemia, monocytic (FAB-M5) leukemia, or the microgranular variant of acute promyelocytic leukemia (FAB-M3) . • Symptoms of leukostasis occur less frequently and typically affect pts with WBC counts over 100 x 109/L. • ALL: • The incidence appears to be highest in infants, patients between the ages of 10 and 20 years, males, and those with a T cell phenotype • Symptoms of leukostasis are rarely seen. • TLS and DIC are more common complications related to the elevated WBC count.
  7. 7. • CLL • Symptoms of leukostasis are rare unless the WBC count exceeds 400 x 109/L. • CML • Pts. with CML typically present with leukocytosis and a median WBC count of approx. 100 x 109/L • Symptoms of leukostasis are very uncommon in chronic phase but can be seen occ. in patients with myeloid blast crisis and very elevated blast counts.
  8. 8. PATHOPHYSIOLOGY OF LEUKOSTASIS • There are two main theories, which are not mutually exclusive:  Large population of leukemic blasts (less deformable than mature leukocytes) Increases blood viscosity Plug microcirculation  impede blood flow (leukostasis).  Worsened by RBC transfusions or the use of diuretics, both of which can increase whole blood viscosity.  Local hypoxemia may be exacerbated by the high metabolic activity of the dividing blasts and the associated production of various cytokines Endothelial damage. • Primitive myeloid leukemic cell are capable of invading through the endothelium and causing hemorrhage. • Brain(40 pc ) and lung(30 pc) are most commonly affected.
  9. 9. CLINICAL FEATURES • Pulmonary involvement • Dyspnea and hypoxia with/out diffuse interstitial or alveolar infiltrates on imaging studies. • Arterial PaO2 can be falsely decreased in pts. with hyperleukocytosis, since the WBCs in the test tube utilize oxygen. • Pulse oximetry provides a more accurate assessment of O2 saturation in this setting. • Neurological involvement • Visual changes, headache, dizziness, tinnitus, gait instability, confusion, somnolence, and, occasionally, coma. • Increased risk of intracranial hemorrhage that persists for at least a week after the reduction of white cell count, perhaps from a reperfusion injury as areas of the brain that were ischemic from leukostasis regain blood flow.
  10. 10. Cont.. • Approx. 80 pc. of pts. with leukostasis are febrile, which may be due to inflammation associated with leukostasis or concurrent infection. • Less common s/s of leukostasis include • ECG evidence of myocardial ischemia or right ventricular overload, • Worsening renal insufficiency, • Priapism, • Acute limb ischemia, or • Bowel infarction
  11. 11. LABORATORY ABNORMALITIES • Pulse oximetry provides a more accurate assessment of O2 saturation. • R/O Pseudo thrombocytosis : Manual counting • R/O Pseudo hyperkalemia • DIC occurs in up to 40 pc. of pts. : FDP and d-Dimer. • Spontaneous TLS is present in up to 10 pc. of pts. with leucostasis : Uric acid, potassium, phosphate, calcium and creatinine.
  12. 12. TREATMENT • For pts with symptomatic or asymptomatic hyperleukocytosis • Initial t/t with induction chemotherapy rather than hydroxyurea or leukapheresis. • Accompanied by TLS prophylaxis with aggressive hydration and allopurinol. • For pts without symptoms of leukostasis who must have induction chemotherapy delayed • Cytoreduction with hydroxyurea rather than leukapheresis. • Cytoreduction with hydroxyurea can ppt. or exacerbate hyperuricemia and/or TLS  IV hydration and TLS prophylaxis. • For pts with symptoms of leukostasis who must have induction chemotherapy delayed • Initial cytoreduction with leukapheresis in combination with hydroxyurea to lower or stabilize the WBC count.
  13. 13. Cont.. • Treatment of the leukemia can result in pulmonary hemorrhage from lysis of blasts in the lung, called leukemic cell lysis pneumopathy. • When APML is treated with differentiating agents like tretinoin and arsenic trioxide • Cerebral or pulmonary leukostasis may occur as tumor cells differentiate into mature neutrophils(Differentiation syndrome). • This complication can be largely avoided by using cytotoxic chemotherapy or arsenic together with the differentiating agents.
  15. 15. INTRODUCTION • MSCC is defined as compression of the spinal cord and/or cauda equina by an extradural tumor mass. • The minimum radiologic evidence for cord compression is indentation of the theca at the level of clinical features. • Spinal cord compression occurs in 5–10% of pts with cancer. • Epidural tumor is the first manifestation of malignancy in about 10% of pts. • Lung cancer is the most common cause of MSCC.
  16. 16. Cont.. • Metastatic tumor involves the vertebral column more often than any other part of the bony skeleton. • Lung, breast, and prostate cancer are the most frequent offenders. • Multiple myeloma also has a high incidence of spine involvement. • Lymphomas, melanoma, renal cell cancer, and genitourinary cancers also cause cord compression. • The thoracic spine is the most common site (70%), followed by the lumbosacral spine (20%) and the cervical spine (10%). • Involvement of multiple sites is most frequent in patients with breast and prostate carcinoma.
  17. 17. • Direct extension of a paravertebral lesion through the intervertebral foramen : Lymphoma, myeloma, or pediatric neoplasm. • Parenchymal spinal cord metastasis due to hematogenous spread is rare. • Intramedullary metastases can be seen in lung cancer, breast cancer, renal cancer, melanoma, and lymphoma.
  18. 18. PATHOPHYSIOLOGY • Obstruction of the epidural venous plexus leads to edema. • Local production of inflammatory cytokines enhances blood flow and edema formation. • Compression compromises blood flow, leading to ischemia. • Production of vascular endothelial growth factor is associated with spinal cord hypoxia.
  19. 19. CLINICAL FEATURES • Localized back pain and tenderness : MC presentation • Involvement of vertebrae by tumor. • Pain is usually present for days or months before other neurologic findings appear. • Exacerbated by movement and by coughing or sneezing. • Radicular pain : Less common • Usually develops later. • Radicular pain in the cervical or lumbosacral areas may be unilateral or bilateral. • Radicular pain from the thoracic roots is often bilateral and is described by pts as a feeling of tight, band-like constriction around the thorax and abdomen.
  20. 20. Cont.. • Lhermitte’s sign • Loss of bowel or bladder control may be the presenting symptom but usually occurs late in the course. • Occasionally pts present with ataxia of gait without motor and sensory involvement due to involvement of the spinocerebellar tract.
  21. 21. PHYSICAL EXAMINATION • Pain induced by SLRT, neck flexion, or vertebral percussion may help to determine the level of cord compression. • Loss of sensation to pinprick is as common as loss of sensibility to vibration or position. • The upper limit of the zone of sensory loss is often one or two vertebrae below the site of compression. • Motor examination • Spastic weakness, spasticity • Extensor plantar reflex and brisk DTR. • Motor and sensory loss usually precedes autonomic dysfunction.
  22. 22. Cont.. • Autonomic dysfunction • Present with decreased anal tonus, decreased perineal sensation, and distended bladder. • The absence of the anal wink reflex or the bulbocavernosus reflex confirms cord involvement. • Residual volume of >150 mL suggests bladder dysfunction.
  23. 23. MIMICKERS • Osteoporotic vertebral collapse, • AIDP, • Pyogenic abscess or vertebral tuberculosis, • Radiation myelopathy, • Neoplastic leptomeningitis, • Benign tumors, • Epidural hematoma, • Spinal lipomatosis.
  24. 24. • Cauda equina syndrome: characterized by • Low back pain • Diminished sensation over the buttocks, posterior-superior thighs, and perineal area in a saddle distribution • Bowel and bladder dysfunction; impotence • Absent bulbo-cavernous and DTR. • Variable amount of lower-extremity weakness. • The majority of cauda equine tumors are primary tumors of glial or nerve sheath origin; metastases are very rare.
  25. 25. IMAGING • IOC • Full-length image of the cord by Contrast MRI • Myelography : Reserved for pts who have poor MRIs or who cannot undergo MRI promptly. • CT Erosion of the pedicles (the “winking owl” sign) is the earliest radiologic finding. • Other radiographic changes include • Increased intra-pedicular distance, • Vertebral destruction, • Lytic or sclerotic lesions, • Scalloped vertebral bodies, and • Vertebral body collapse.
  26. 26. MANAGEMENT • Goal of therapy • Relief of pain • Restoration/preservation of neurologic function • Radiation therapy plus glucocorticoids is generally the initial t/t of choice. • Up to 75% of pts treated when still ambulatory remain ambulatory, but only 10% of patients with paraplegia recover walking capacity.
  27. 27. • INDICATIONS FOR SURGICAL INTERVENTION • Unknown etiology, • Failure of radiation therapy, • Radioresistant tumor type (e.g., melanoma or renal cell cancer), • Pathologic fracture dislocation, and • Rapidly evolving neurologic symptoms
  28. 28. • TYPES OF SURGERY • Laminectomy • Posteriorly localized epidural deposits in the absence of vertebral body disease. • Resection of the anterior vertebral body along with the tumor, followed by spinal stabilization • Anterior or anterolateral epidural abscess. • A RCT showed that pts who underwent surgery followed by radiotherapy (within 14 days) retained the ability to walk significantly longer than those treated with radiotherapy alone.
  29. 29. • Chemotherapy may have a role in pts with chemosensitive tumors who have had prior radiotherapy to the same region and who are not candidates for surgery. • Patients with metastatic vertebral tumors may benefit from percutaneous vertebroplasty or kyphoplasty, the injection of acrylic cement into a collapsed vertebra to stabilize the fracture. • Pain palliation is common. • Cement leakage seen in 10% of pts.
  31. 31. Cont..
  33. 33. • Seen with initial infusion of human or humanized antibodies (e.g., rituximab, gemtuzumab, trastuzumab, alemtuzumab, panitumumab, brentuximab vedotin) • A/w fever, chills, nausea, asthenia, and headache in up to half of treated pts. • Bronchospasm and hypotension occur in 1% of pts. • Severe manifestations including pulmonary infiltrates, ARDS , and cardiogenic shock occur rarely.
  34. 34. • Lab manifestations include elevated hepatic aminotransferase levels, thrombocytopenia, and prolongation of PT. • Pathogenesis • Activation of immune effector processes (cells and complement) release of inflammatory cytokines, s/a TNF-α, INF-gamma, IL-6, and IL-10 (Cytokine Release Syndrome[CRS]). • Treatment • Diphenhydramine, hydrocortisone, and acetaminophen can often prevent or suppress the infusion-related symptoms. • If they occur, the infusion is stopped and restarted at half the initial infusion rate after the symptoms have abated. • Severe CRS may require intensive support for ARDS and resistant hypotension.
  36. 36. INTRODUCTION • Neutropenic enterocolitis (Typhlitis) is the inflammation and necrosis of the cecum and surrounding tissues that may complicate the treatment of acute leukemia. • Nevertheless, it may involve any segment of the GIT including small intestine, appendix, and colon. • Clinical presentation • Right lower quadrant abdominal pain, often with rebound tenderness and a tense, distended abdomen, in a setting of fever and neutropenia. • Watery diarrhea (often containing sloughed mucosa)along with bleed. • Bacteremia
  37. 37. DIAGNOSIS • CT SCAN • Marked bowel wall thickening, particularly in the cecum, • Bowel wall edema, • Mesenteric stranding, and • Ascites • Patients with bowel wall thickness >10 mm on USG have higher mortality rates. • Pneumatosis intestinalis is a more specific finding, seen only in those with neutropenic enterocolitis and ischemia. • The combined involvement of the small and large bowel suggests a diagnosis of neutropenic enterocolitis
  38. 38. TREATMENT • Rapid institution of broad-spectrum antibiotics, bowel rest, and nasogastric suction. • Surgical intervention  Reserved for severe cases of neutropenic enterocolitis with  Evidence of perforation,  Peritonitis,  Gangrenous bowel, or  GI bleed despite correction of any coagulopathy
  39. 39. REFERENCE • Harrison 19th Edition • Wintrobe’s clinical hematology 13th edition • UpToDate 2018
  40. 40. THANK YOU