Hematologic emergencies and Transfusions

1. HOSAM ATEF;MD
SUEZ CANAL UNIVERSITY
FACULTY OF MEDICINE
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2. 2

3. • Lymphadenopathy: mostly above diaphragm
(lower neck, subclavicular)
• Large mediastinal mass  chest pressure,
cough, dyspnea
• B symptoms: weight loss, fever, night sweats
– May be presenting complaint
Lymphoma: Hodgkin
3

4. • Diagnosis: lymph node biopsy showing Reed-
Sternberg cells
• Treatment: chemotherapy / radiation
Lymphoma: Hodgkin
4

5. • Proliferation of lymphoid cells from lymph
nodes OR lymphatic tissue other than bone
marrow
• B-Cell, T-Cell, NK-Cell: can be indolent or
aggressive
• Diagnosis: biopsy
• Treatment: chemotherapy / radiation
Non-Hodgkin Lymphoma
5

6. • Anemia + thrombocytopenia + neutropenia
• Bone marrow diseases: myelo-dysplasia,
myelofibrosis, some leukemias and
lymphomas
• Systemic diseases: lupus, severe infection,
sarcoidosis, alcohol, tuberculosis, vitamin
B12, folate
Pancytopenia
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7. 7

8. • Pancytopenia with hypocellular bone marrow
– Acquired: marrow stem cells damaged by
drugs, radiation, virus, chemical, immune-
related
– Inherited: Fanconi’s anemia, dyskeratosis
congenita
Aplastic Anemia
8

9. • Bleeding, easy bruising
• Fatigue, malaise, anemia  short of breath
• Infection common
Signs & Symptoms
9

10. • CBC, reticulocyte count
• Bone marrow biopsy
• Immediate: depends on severity of anemia /
thrombocytopenia / neutropenia ±
complications
• Bone marrow transplant
Diagnosis / Treatment
10

11. Red Blood Cells (RBC)
• Normal life: ~120 days
• Marrow releases ~1% total count daily
– “baby red cells” = nucleated =
reticulocytes
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12. 7.The most helpful laboratory study to differentiate
poor red blood cell production from increased red
cell destruction is the:
a. sedimentation rate.
b. sideroblast level.
c. serum iron level.
d. total to direct bilirubin ratio.
e. reticulocyte count.
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13. 7.The most helpful laboratory study to differentiate
poor red blood cell production from increased red
cell destruction is the:
a. sedimentation rate.
b. sideroblast level.
c. serum iron level.
d. total to direct bilirubin ratio.
e. reticulocyte count.
13

14. 7.  production vs  destruction
• Reticulocytes are RBCs of intermediate maturity.
•  reticulocyte count reflects impaired RBC
production; seen with low levels of iron, vitamin
B12, folate, bone marrow failure.
•  reticulocyte count reflects accelerated
erythropoeisis, the normal marrow response to
anemia; seen with blood loss and hemolytic
anemias. 14

15. Aplastic Anemia
• Normal MCV with  reticulocyte
• Can be RBC aplasia alone
15

16. Sickle Cell Disease
• Inherited mutated hemoglobin: HbS
• Recessive gene: both parents need to pass
to offspring
• HbS + HbA: trait  asymptomatic
• HbS + HbC (beta-thalassemia): chronic
anemia + recurrent painful crises
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17. Sickle Cell Disease
• RBC life span 10 – 15 days
• Marrow on constant “overdrive”
• Reticulocyte “normal” 8 – 10%
• WBC “normal” at 15 – 20K
• Hemoglobin “normal” at 8 – 10
• Platelets “normal” >500K
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18. Vaso-Occlusive Crisis
• AKA acute painful episode
• Pain in extremities, chest, abdomen, back
• No lab test can confirm
• No vital sign can confirm
• Treatment: analgesia
– Avoid meperidine
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19. Acute Chest Syndrome
• New infiltrate on chest x-ray + fever, cough,
sputum, dyspnea, tachypnea, hypoxia
• Many causes: fat embolism from marrow
ischemia, pulmonary vaso-occlusion,
infection, venous thromboembolism,
pulmonary edema
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20. Acute Chest Syndrome
• Children: fever & cough most common
• Adults: fever & chest pain as presenting
complaint
– Some develop 2 – 3 days into hospital stay
• ± hypoxemia
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21. Acute Chest Syndrome
• Chest x-ray normal in ~50%
• Treatment supportive
• Empiric antibiotic: 3rd / 4th generation
cephalosporin + macrolide
• Severe  exchange transfusion
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22. Splenic Sequestration
• Etiology: unknown
• Typically occurs in infants
– Tender, enlarged spleen
– Signs of hypovolemia, shock
– Concurrent infection common
• Diagnosis: worsening anemia, persistent
reticulocytisis, tender large spleen
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23. Splenic Sequestration
• Treatment:
– Intravenous fluids
– Blood transfusion
– May need splenectomy after acute episode
resolves
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24. Aplastic Crisis
• Marrow shuts down
• Abrupt  hemoglobin
• Abrupt  reticulocytes (<2%)
• Causes: folate deficiency, human parvovirus
B19 (in children)
• Blood transfusion: severe anemia,
cardiorespiratory symptoms
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25. Hemolytic Crisis
• Abrupt  hemoglobin
• Major increase reticulocyte production
• May see worsening jaundice
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26. Other Complications
• Functional asplenia: infected by encapsulated
organisms
• Strokes: CNS occlusive events
• Priapism: low-flow due to corpora cavernosa
occlusion
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27. 2.The viral agent implicated in an aplastic crisis of
patients with sickle cell disease is:
a. adenovirus (atypical).
b. herpes simplex.
c. parvovirus.
d. coxsackie virus.
e. HTLV-IV.
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28. 2.The viral agent implicated in an aplastic crisis of
patients with sickle cell disease is:
a. adenovirus (atypical).
b. herpes simplex.
c. parvovirus.
d. coxsackie virus.
e. HTLV-IV.
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29. 2.Parvovirus & Sickle cell
Aplastic crises can be precipitated by viral
infections (particularly parvovirus B19), folic acid
deficiency, or the ingestion of bone marrow toxins
such as phenylbutazone. Bone marrow
erythropoiesis is slowed or stopped. The
hematocrit falls to as low as 10%, and the
reticulocyte count falls to as low as 0.5%. The
white blood cell count and platelet counts usually
remain stable.
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30. 9.A 12-year-old girl with sickle cell disease is
brought by her mother after she passed out twice.
She was kept home from school the last few days
for a “cold.” When you ask the child to stand, you
must catch her to prevent her from falling to the
ground. This is suspicious for:
a. salmonella sepsis.
b. sequestration crisis.
c. acute chest syndrome.
d. aplastic crisis.
e. hemolytic crisis.
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31. 9.A 12-year-old girl with sickle cell disease is
brought by her mother after she passed out twice.
She was kept home from school the last few days
for a “cold.” When you ask the child to stand, you
must catch her to prevent her from falling to the
ground. This is suspicious for:
a. salmonella sepsis.
b. sequestration crisis.
c. acute chest syndrome.
d. aplastic crisis.
e. hemolytic crisis.
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32. 9.Sequestration crisis
Sequestration crisis occurs primarily in children
and is the second most common cause of death in
children with SCD under the age of 5 years. Often
preceded by viral infections, sickled cells block the
splenic outflow, causing pooling of peripheral
blood and sickled cells in the spleen. Such
patients present in hypovolemic shock with an
enlarged spleen.
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33. Hemolytic Anemia
• RBC destruction
• Intravascular
– Smear  fragmented RBCs (schistocytes)
– More acute than extravascular
• Extravascular
– Occurs in liver or spleen
– Smear  spherocytes
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34. Hemolytic Anemia
• Intrinsic
– Enzyme defect (G6PD, pyruvate kinase),
abnormal membrane, abnormal cell (sickle
cell disease, thalassemia)
– G6PD triggered by drugs (aspirin,
antimalarials, sulfa, nitrofurantoin,
phenazopyridine), foods (fava beans),
infection 34

35. Hemolytic Anemia
• Extrinsic
– Autoimmune
– Non-immune: HUS/TTP, HELLP, prosthetic
valve abnormality, arterio-venous
malformation, malaria, drugs, spider /snake
venom
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36. Signs & Symptoms
• Vary depending on disease
• Symptoms related to anemia
• Jaundice
• Hepatosplenomegaly
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37. Diagnosis
• Smear  schisto-, spherocytes
• Reticulocytes should be 
• Haptoglobin: binds free Hgb 
• Bilirubin 
• LDH  (released from RBCs)
• Hemoglobinemia, hemoglobinuria
• Autoimmune  direct Coombs +
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38. Hypochromic Microcytic Anemia
•  iron  iron deficiency
•  globin  thalassemia
•  porphyrin  sideroblastic, lead toxicity
• Chronic disease
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39. 13. A healthy 12-year-old African-American
female complains of weakness and fatigue 3
days after starting a course of trimethoprim-
sulfamethoxasole and pyridium for a urinary
tract infection. Her hemoglobin is 4.8 mg/dl,
and her urine is tea-colored, but you see no
red blood cells on microscopic exam. She
probably has undiagnosed:
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40. 13.12-year-old African-American
female…weakness and fatigue… TMP/SMZ …
hgb 4.8 mg/dl … no RBCs in urine. Undiagnosed:
a. hemolytic uremic syndrome.
b. G6PD deficiency.
c. idiopathic thrombocytopenic purpura.
d. thrombotic thrombocytopenic purpura.
e. sickle cell disease.
40

41. 13.12-year-old African-American
female…weakness and fatigue… TMP/SMZ …
hgb 4.8 mg/dl … no RBCs in urine. Undiagnosed:
a. hemolytic uremic syndrome.
b. G6PD deficiency.
c. idiopathic thrombocytopenic purpura.
d. thrombotic thrombocytopenic purpura.
e. sickle cell disease.
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42. 13.G6PD
• Deficiency of the RBC enzyme glucose-6-
phosphate dehydrogenase (G-6-PD) is the most
common human enzyme defect, affecting nearly
one-tenth of the world’s population
• The RBC is unable to protect itself against
oxidant stress. Acute hemolytic crises occur
that are incited by bacterial and viral infections,
exposure to oxidant drugs, metabolic acidosis
(such as diabetic ketoacidosis), and ingestion of
fava beans in some patients.
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43. 13.G6PD
Within 1 to 3 days following oxidant stress, the
patient can develop hemoglobinuria and the
potential for vascular collapse. These hemolytic
crises are generally well tolerated and self-limited
because only the older RBCs will hemolyze. The
drugs most commonly associated with oxidant
stress are sulfa drugs, antimalarials,
phenazopyridine, and nitrofurantoin.
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44. Diagnosis / Treatment
•  serum iron level
•  serum ferritin level
•  total iron binding capacity (TIBC)
• Treatment: iron supplements, outpatient
workup
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45. Macrocytic Anemia
• Most important: megaloblastic
•  folate (green vegetables, fruit, cereals) or
 vitamin B12 (meat)  impaired DNA
synthesis
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46. Macrocytic Anemia
• Petechiae, mucosal bleeding, infections, sore
mouth / tongue, diarrhea, weight loss
• B12 can also have paresthesias, ataxia
• Diagnosis: send levels
• Treatment: replace
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47. Polycythemia
• Hct: >51% in M, >48% in F
• Apparent:  plasma volume
• Secondary
– Appropriate: hypoxia
– Inappropriate:  erythropoietin
• Polycythemia vera:  production RBCs,
WBCs, platelets
– Can progress to myelofibrosis, leukemia
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48. Signs & Symptoms
• Headache, weakness, pruritus, dizziness,
sweating, visual changes, paresthesias,
weight loss, joint pain
• Ruddy complexion, spleen, liver, blood
pressure
• Thrombosis: ACS, CVA, PE, etc
• Hemorrhage
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49. Diagnosis / Treatment
• RBCs, WBCs, platelets
• Confirmed by non-ED studies
• Treatment: phlebotomy
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50. 16. A 68-year-old man complains of headache,
dizziness, and blurred vision. His blood
pressure is 190/118 mmHg. He has a florid
face, normal fundi, and marked
splenomegaly. His hematocrit is 67%.
Reasonable therapy includes:
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51. 16. … florid face … splenomegaly …
hematocrit = 67%. Therapy:
a. 250cc salt-poor albumin.
b. intravenous nitroprusside.
c. phlebotomy.
d. plasmapheresis.
e. sublingual nifedipine.
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52. 16. … florid face … splenomegaly …
hematocrit = 67%. Therapy:
Emergency treatment of any form of symptomatic
polycythemia is phlebotomy. Usually not more
than 500 ml of blood is slowly removed as the
volume is replaced with a comparable amount of
normal saline.
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53. Methemoglobinemia
• production or reduction
• Causes discussed in toxicology
• Cyanosis with normal oxygen saturation
• Treatment:
– Congenital: usually none
– Toxic  symptomatic  treat with
intravenous methylene blue
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54. 54

55. Leukemia
• production undifferentiated hematopoietic
stem cells
• Acute Lymphocytic (ALL)
• Signs & symptoms: protean
– Related to abnormal cells crowding out
normal cells  RBC, WBC, platelets
– Bacterial infections in 1/3 at time of
diagnosis 55

56. Leukemia
• Chronic Lymphocytic (CLL): most common in
patients >50 years
• Often no symptoms: may have fatigue, large
lymph nodes, infections (esp. respiratory)
• Diagnosis: absolute lymphocyte count >5000
cells/ml
• Treatment: monitor, chemo
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57. Multiple Myeloma
• Abnormal plasma cells
• Fatigue from anemia
• Bone pain from osteolytic lesions or
pathologic fractures
• Diagnosis: calcium, creatinine
• Bone marrow: >10% plasma cells
• Serum / urine protein electro-phoresis (SPEP,
UPEP) 57

58. Treatment
• Chemotherapy
• Plasmapheresis for hyperviscosity syndrome
• If comatose: temporize by removing 1 liter
blood, replace with normal saline
• calcium: IV saline, steroids
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59. 59

60. Malignancy Complications
• Hyperviscosity syndrome
• Leukostasis (sludging)
• Neutropenic fever
• Spinal cord compressions
• Superior vena cava syndrome
• Tumor lysis syndrome
• Hypercalcemia
• Pericardial effusion / tamponade 60

61. Hyperviscosity Syndrome
• abnormal serum proteins
• Waldenstrom macroglobulinemia
• Multiple myeloma (less common)
• Most common symptoms: neurologic, visual
• May see mucosal or GI bleeding
• CHF from  plasma volume
• Plasmapheresis, exchange
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62. Leukostasis
• WBC sludging in microcirculation
• Usually acute leukemia
• Can be seen with chronic, NHL
• Neurologic symptoms
• Can see respiratory failure
• Treatment: leukapheresis, hydroxyurea,
chemotherapy
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63. Neutropenic Fever
• Absolute neutrophil count (ANC) =
neutrophils + bands
• ANC <500 cells/ml
• Signs and symptoms: fever
• Treatment: IV antibiotics
– Ceftazidime or cefepime ± amino-glycoside
or –penem
– Add vancomycin if appropriate 63

64. Spinal Cord Compression
• Lymphoma, metastasis, primary
• S&S: back pain, weakness, numbness, bowel
/ bladder / sexual dysfunction
• Diagnosis: MRI
– CT myelogram if MRI not available
• Treatment
– Dexamethasone
– Radiation therapy 64

65. Superior Vena Cava Syndrome
• Tumor obstructs SVC  venous
hypertension, congestion
• Early signs: face edema that improves
through day
• SOB, cough, chest pain
• Distension of chest, neck veins
• Cyanosis
• Diagnosis: chest CT
• Treatment: radiation therapy
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66. Acute Tumor Lysis Syndrome
• Usually after chemotherapy
• Lysed cells  metabolic changes
• Hyperuricemia: N/V, renal failure due to renal
precipitation
• Hyperphosphatemia: N/V, lethargy, seizures,
renal impair
– Also binds with Ca+  calcium
• Hypocalcemia: tetany, arrhythmia 66

67. Acute Tumor Lysis Syndrome
• Hyperkalemia: N/V, muscle weakness,
cramps, arrhythmias, heart block  asystole
• Acute renal failure: possible hemodialysis; do
NOT alkalinize urine, worsens phosphorus
and calcium
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68. Others
• Hypercalcemia: see Endocrine-Metabolic
Emergencies
• Pericardial Effusion / Tamponade: see
Cardiovascular Emergencies
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69. 12.A 42-year-old woman with adult T-cell
lymphoma-leukemia complains of back pain,
abdominal pain, and confusion. Laboratory
evaluation shows a total calcium of 15.8 mg/dl.
Appropriate management of this patient should
include:
a. plasmapheresis.
b. IV bicarbonate.
c. IV hypertonic saline / oral Kayexalate®.
d. IV normal saline and IV furosemide.
e. glucagon.
69

70. 12.A 42-year-old woman with adult T-cell
lymphoma-leukemia complains of back pain,
abdominal pain, and confusion. Laboratory
evaluation shows a total calcium of 15.8
mg/dl. Appropriate management of this
patient should include:
a. plasmapheresis.
b. IV bicarbonate.
c. IV hypertonic saline / oral Kayexalate®.
d.IV normal saline and IV
furosemide.
e. glucagon.
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71. 12.Hypercalcemia of malignancy
Patients with severe hypercalcemia (>14 mg/dl)
require immediate treatment regardless of
symptoms. The four basic goals of therapy are
(1)restore intravascular volume,
(2)enhance renal calcium elimination
(3)reduce osteoclastic activity
(4)treat primary disorder.
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72. 12.Hypercalcemia of malignancy
Isotonic saline is the first step. Once volume is
restored, the calcium will usually have  by 1.6 to
2.4 mg/dl, but hydration alone rarely leads to
complete normalization. Loop diuretics inhibit
resorption of calcium in the thick ascending loop of
Henle,  the calciuric effect of hydration. Volume
expansion must precede administration of
furosemide, because the drug’s effect depends on
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73. Pearls
• The first step in managing any transfusion
reaction to to stop the transfusion
• Hemarthrosis in hemophiliacs: factor
replacement, never arthrocentesis
• Minor head injury in hemophilia: replace
factor, head CT, admit for observation
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74. Pearls
• Initial treatment in mild-moderate hemophilia
A: DDAVP
• NO PLATELET TRANSFUSION in patients
with TTP, HUS
• Once a patient has HIT, no heparin can ever
again be used, including LMWH
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75. Thank
You
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