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hematology Flashcards

1
Q

associated condition with aplastic anemia and what conditions are patients with AA susceptible to

A

have an associated thymoma

and have an increased risk of developing MDS or leukemia

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2
Q

what causes PNH

A

results from a genetic mutation of membrane proteins that ameliorate complement-mediated destruction of erythrocytes

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3
Q

signs of PNH

A
  • chronic hemolytic anemia
  • iron deficiency through urinary losses
  • venous thrombosis (including Budd-Chiari syndrome)
  • pancytopenia
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4
Q

test for aplastic anemia/ PNH

A

bone marrow aspirate and biopsy (hypocellular with
increased fat content)
• cytogenetic analysis to exclude other bone marrow disor-
ders (e.g., MDS)
• PNH screening flow cytometry with cell surface markers
CD55 and CD59 absent
• vitamin B12 and folate levels, hepatitis serologies, and HIV
testing

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5
Q

Tx for aplastic anemia and PNH

A
  • stop potential causative agent
  • first line is cyclosporine followed by HCST
  • PNH first line is eculizumab, long term do HSCT and dont forget to start AntiCoagulation and supplement repletion of folic acid and iron
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6
Q

what is pure red cell aplasia

A

absence or a marked decrease of erythrocyte production with

normal leukocyte and platelet counts.

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7
Q

causes of red cell aplasia

A

T cell autoimmunity (pregnancy, thymoma, malignancy) or

direct toxicity to erythrocyte precursors (viral infection, drug toxicity).`

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8
Q

diagnsosis of red cell aplasia

A

Bone marrow shows profound erythroid hypoplasia. Clonal CD57-positive T cells consistent with large granular lymphocytosis
are often found.

Get CT to rule out thymoma

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9
Q

tx of red cell aplasia

A
  • transfusion support and immunosuppressive drugs (prednisone, cyclosporine, antithymocyte globulin)
  • thymectomy for thymoma
  • IV immune globulin for patients with AIDS and chronic parvovirus B19 infection
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10
Q

isolated neutropenia causes

A

Infectious
HIV CMV EBV rickettsial

Drugs
Chemo, NSAIDs, carbamazepine, phenytoin, propylthiouracil, cephalosporins, trimethoprim-sulfamethoxazole

AutoImmune
RA SLE Felty syndrome (RA splenomegaly neutropeni)

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11
Q

what is MDS

A

clonal disorders of the hematopoietic stem cells that occur predominantly in patients older than 60 years and are characterized by ineffective hematopoiesis and peripheral cytopenias.

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12
Q

what will MDS lead to

A

acute leukemia syndrome or death

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13
Q

bone marrow finding in mds

A

hypercellular with dysplastic erythroid precursors

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14
Q

diagnosis of mds

A

Look for cytopenia in at least two
lines (anemia, leukopenia, thrombocytopenia) and morphologic abnormalities of erythrocytes (macrocytosis with nucleated
erythrocytes and teardrop cells).

Patients could present with only anemia, normal b12 and folate and high MCV

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15
Q

MDS clonal abnormalities involve

A

involving chromosomes 3, 5, 7, 8, and 17 supports the diagnosis. Look for −5q
syndrome, a subtype of MDS that has a specific therapy.

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16
Q

treatment for mds

A

low risk- no tx
potentially can use esa to avoid frequent transfusions
high risk- need tx to avoid aml

Allogeneic HSCT is offered to fit younger patients and azacytidine and decitabine to persons at high or very high risk for AML

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17
Q

treatment for -5q syndrome

A

lenalidomide

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18
Q

CML translocation

A

9:22 chromosome Philadelphia

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19
Q

what are patients with CML susceptible to

A

transitioning to AML- blast crisis

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20
Q

signs of cml

A

asymptomatic patients are splenomegaly, an elevated leukocyte count, and an increased number of
granulocytic cells in all phases of maturation on the peripheral blood smear.

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21
Q

how do you know if patient is in blast phase

A

> 20% of leukocytes are blast

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22
Q

Tx of CML
palliative
maintenance
cure

A

Hydroxyurea- Palliative, only to alleviate leukocytosis and splenomegaly

Tyrosine kinase inhibitors: imatinib mesylate, dasatinib,
and nilotinib- Disease control with life long tx

Allogeneic HSCT- cure for some with accelerated (10-20% or blast (>20%) disease

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23
Q

what testing needs to be done with tyrosine kinase inh

A

ekg because prolonged qt

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24
Q

what is essential thrombocytemia

A

predominant increase in megakaryocytes and platelet counts greater than 450,000/μL in the absence of
secondary causes

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25
Q

if patient were to have symptoms of essential thrombocytemia what would they be

A
  • vasomotor disturbances such as erythromelalgia (red and painful hands or feet with warmth and swelling)
  • livedo reticularis
  • headache
  • vision symptoms
  • arterial or venous thromboses
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26
Q

diagnosis of ET

A

splenomegaly and Jak 2 mutation (50%)

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27
Q

tx of ET

A

Low-risk patients (age <60 years, no previous thrombosis, leukocyte count <11,000/μL) may be treated with low- dose aspirin, which reduces vasomotor symptoms.
High-risk nonpregnant patients are treated with hydroxyurea in addition to aspirin.

Plateletpheresis is used when the platelet count must be reduced quickly in life-threatening situations such as TIA, stroke, MI,
or GI bleeding.

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28
Q

most common causes of thrombocythemia

A

iron deficiency and infection

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29
Q

how to differentiate primary vs secondary polycythemia vera

A

primary has low to normal EPO
secondary - elevated erythropoietin level, although a markedly elevated erythropoietin level suggests
ectopic production by a renal cell cancer or other kidney disease.

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30
Q

secondary polycythemia vera

A

hypoxemia (most
common), volume contraction because of diuretics, use of androgens, and secretion of erythropoietin by kidney or liver
carcinoma.

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31
Q

symptoms of polycythemia vera

A

thrombosis or bleeding, facial plethora, erythromelalgia, pruritus exacerbated by bathing in hot
water, and splenomegaly.

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32
Q

dx of polycythemia

A

Patients with PCV have a low serum erythropoietin level in the setting of erythrocytosis.

An activating mutation of JAK2 is present in 97% of patients with PV.

Microscopic hematuria may be the only sign of an erythropoietin-producing hypernephroma as the cause of an elevated
hemoglobin and erythrocyte count.

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33
Q

tx for polycythemia vera

A

Therapeutic phlebotomy should be instituted with the goal of lowering the hematocrit level to <45%.

Hydroxyurea in addition to phlebotomy is often the treatment of choice for patients at high risk for thrombosis (e.g., >60 years,
previous thrombosis, leukocytosis).

Low-dose aspirin is indicated unless strong contraindications exist.

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34
Q

Hepatic vein thrombosis (the Budd-Chiari syndrome) or portal vein thrombosis should prompt evaluation for

A

PV

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35
Q

cut off for hypereosinophic syndrome

A

eosinophil >1500, infiltrates in the liver, spleen, heart, and lymph
nodes; and systemic symptoms

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36
Q

causes of eosinophilia (5)

A

Collagen vascular disease (eosinophilic granulomatosis with
polyangiitis is prototypical)

Helminthic (parasitic worm) infection

Idiopathic (no cause after extensive investigation)

Neoplasia (lymphomas are most common)

Allergy, atopy, asthma

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37
Q

clinical features of ALL

A

rapidly rising blast
cells in the blood and bone marrow, bulky lymphadenopathy (especially in the mediastinum), a younger age at onset, and cytopenia secondary to bone marrow involvement. Up to 30% of patients with ALL have CNS involvement

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38
Q

tx for ALL

A 
Combination chemotherapy often followed by allogeneic HSCT.
CNS prophylaxis (intrathecal chemotherapy with or without radiation) is also indicated.

If Philadelphia chromosome is present (9:22) can treat with imatinib along with above

39
Q

signs of AML

A

elevated leukocyte count, anemia, thrombocytopenia, and blasts on peripheral blood
smear

Gingival hypertrophy and leukemia cutis (violaceous, nontender cutaneous plaques) are commonly encountered.
Pathognomonic Auer rods may be seen on a peripheral blood smear.

seldom with lymphadenopathy and splenomegaly

40
Q

diagnosis of aml

A

bone marrow aspiration and biopsy showing >20% myeloblasts.

41
Q

what is apl

A

special case marked by the t(15;17) translocation, which disturbs a retinoic acid receptor.
Patients with acute promyelocytic leukemia have significant bleeding because of fibrinolysis and DIC.

42
Q

tx for APL

A

all-trans retonoic acids

43
Q

what are patient who take ATRA at risk for and how does it present and what is the tx

A

g differentiation syndrome. Characteristic findings are fever, pulmonary infiltrates, hypoxemia, and, occasionally, hyperleukocytosis. Treatment is dexamethasone.

44
Q

when to do plt transfusion and leukophoresis in AML

A

plt transfusion if plt <10k

leukophoreiss >50k

45
Q

normocytic anemia causes based on reticulocyte

A

increased: either blood loss (bleeding or hemolysis) or response to therapy

decreased/normal- under production

  • inflammation with deficient erythropoietin (most frequent cause)
  • nutritional deficiencies (iron, folate, cobalamin)
  • hypometabolism (hypothyroidism, testosterone deficiency)
  • a primary hematopoietic disorder (pure red cell aplasia or myelodysplasia)
46
Q 
iron deficiency anemia 
serum iron 
ferritin 
TIBC
transferrin sat %
A

Serum iron Low
Ferritin Low
TIBC High
Transferrin saturation Low (<10%)

47
Q 
Inflammatory anemia 
serum iron 
ferritin 
TIBC 
transferrin saturation
A

Serum iron Low
Ferritin High
TIBC Low
Transferrin saturation Low/Normal

48
Q

most common cause of microcytic anemia

A

menstrual or GI blood loss or malabsorption

syndromes (celiac disease). Other causes include inflammatory disorders and lead intoxication

49
Q

childhood microscopic iron deficiency should be evaluated for

A

thalassemia trait, other hemoglobinopathies (thalassemia), or ineffective
erythropoiesis (hereditary sideroblastic anemia).

50
Q

signs of iron deficiency anemia

A

restless legs syndrome, hair loss, and spoon nails

51
Q

causes of macrocytic anemia

A
  • folate and/or cobalamin deficiencies
  • drugs affecting folate metabolism and/or DNA synthesis (alcohol, zidovudine, hydroxyurea, methotrexate)
  • acquired causes of megaloblastic maturation such as the MDS
52
Q

testing for macrocytic anemia and what to do if inconclusive results

A

Test B12/Folate
If serum vitamin B12 levels are borderline low (200-300 pg/mL), measure serum methylmalonic acid and homocysteine levels.

Elevated levels confirm vitamin B12 deficiency; elevated homocysteine and normal methylmalonic acid levels are associated
with folate deficiency

53
Q

tx for b12 deficiency

A

High-dose oral vitamin B12 supplementation of 1000 to 2000 μg/d is usually as effective as parenteral administration and should
be the initial therapy for most patients unless cant absorb or swallow orally

54
Q

tx for folate deficency

A

Folate deficiency can be treated with oral folic acid, 1 to 5 mg/d, until complete hematologic recovery; oral therapy is effective
even in malabsorption conditions.

55
Q

classic findings in hemolytic anemia

A

anemia, splenomegaly, elevated reticulocyte count, elevated LDH and indirect bilirubin, decreased
haptoglobin, and elevated MCV

56
Q

examples of congenital hemolytic anemia

A

sickle cell disorders of the erythrocyte membrane (hereditar spherocytosis), enzyme defects (glucose-6-phosphate dehydrogenase deficiency), and thalassemia syndromes.

57
Q

examples of acquired hemolytic anemia

A

o medications (fludarabine, bendamustine, quinine, penicillins, α-methyldopa); can be immune in nature; or can occur secondary to micro- or macroangiopathic processes, infections, or physical agents.

58
Q

Schistocytes and thrombocytopenia

A

TTP-HUS, DIC, HELLP

59
Q

Schistocytes in a patient with a prosthetic heart valve

A

Valve leak

60
Q

Erythrocyte agglutination

A

Cold agglutinin hemolysis (Mycoplasma infection, lymphoproliferative
diseases, CLL)

61
Q

Spherocytosis

A

Autoimmune hemolytic anemia or hereditary spherocytosis

62
Q

Target cells

A

Thalassemia, other hemoglobinopathy, or liver disease

63
Q

Bite cells

A 
G6PD deficiency (suggested by eccentrically located hemoglobin confined
to one side of the cell)
64
Q

Warm autoimmune hemolytic anemia tx

A

initial therapy is glucocorticoids. Alternative agents are available for patients unresponsive to glucocorticoids or splenectomy.

65
Q

Cold agglutinin disease tx

A

primary therapy is cold avoidance or rituximab for persistent symptoms; glucocorticoids or splenectomy are usually ineffective

66
Q

ttp tx

A

emergent plasma exchange.

67
Q

Hereditary spherocytosis and transfusion-dependent thalassemias tx

A

splenectomy

68
Q

Severe thalassemia tx

A

HSCT is standard therapy.

69
Q

Severe PNH tx

A

eculizumab or HSCT

70
Q

A personal or family history of anemia, jaundice, splenomegaly, or gallstones suggests

A

hereditary spherocytosis

71
Q

vaccines needed for sickle cell and hemolytic anemia dz

A

pneumococcal (both 23- and 13-valent), Haemophilus

influenzae type B, influenza, and meningococcal vaccinations.

72
Q

signs of an aplastic crisis

A

hemoglobin drops >2

  • caused by parvovirus b19 or other cytoxic drugs
  • reticulocyte count low
73
Q

Sickle and Chronic pain involving hips and shoulders

A

Osteonecrosis (avascular necrosis)

74
Q

sickle and CVAs

A

Ischemic infarction in children and hemorrhage in adults

75
Q

sickle and Infection with encapsulated organisms

A

Functional asplenia

76
Q

Sickle cell and Liver disease think

A

Viral hepatitis, iron overload from transfusions, or ischemic-induced hepatic
crisis

77
Q

sickle cell and impotence think

A

Prolonged or repeated episodes of priapism

78
Q

Sickle cell and proteinuria or isothenuria think

A

ckd

79
Q

sickle cell and decreased visual acuity

A

retinopathy

80
Q

initial management of vaso-occlusive disease

A

hydration, supplemental oxygen for hypoxemia, treatment of any precipitating event, and
opioids.

81
Q

tx options for sickle cell

A

• Hydroxyurea is used for patients with more than two pain crises each year or for those with ACS to prevent future episodes

Exchange transfusion is indicated for patients with an acute stroke, fat embolism, or ACS.
Use prophylactic exchange transfusion for patients with a history of ischemic stroke.

HSCT should be considered for patients with severe symptoms unresponsive to transfusions and hydroxyurea or endorgan damage.

82
Q

transfusion threshold for sickle cell

A

The transfusion target is hemoglobin level <10 g/dL (hemoglobin
A level >70%).

Do not transfuse patients with simple vasoocclusive pain

83
Q

(–α/αα) [single-gene deletion] clinical syndrome and tx

A

Silent carrier state that is clinically normal

tx: None

84
Q

(– –/αα; or –α/–α) [two-gene deletion] clinical syndrome and tx

A

α-Thalassemia trait; mild microcytic anemia; normal or elevated erythrocyte count; normal hemoglobin electrophoresis

Tx: None

85
Q

(– –/–α) [three-gene deletion] clinical syndrome and tx

A

Hemoglobin H (β4); severe anemia and usually early death Intermittent transfusion

86
Q

(– –/– –) [four-gene deletion] clinical syndrome and tx

A

Hydrops fetalis; fetal death In utero transfusion

87
Q

pathophys of b thalassemia

A

Decreased β-chain synthesis leads to impaired production of hemoglobin A (α2β2) and resultant increased synthesis of hemoglobin A2 (α2δ2) and/or hemoglobin F (α2γ2).

88
Q

β-Thalassemia major

(Cooley anemia) characteristics and tx

A

Two-gene deletion leading to either no production or
severely limited production of β-globin

Tx
Transfusion, iron chelation; consider
splenectomy and HSCT

89
Q

β-Thalassemia minor

(β-thalassemia trait) characteristics and tx

A

A single β-gene leading to reduced β-globin production
with no or mild anemia

Tx
None

90
Q

how to differentiate iron deficiency anemia and thalassemia

A

thalassemia has normal or high ferritin erythrocyte and RDW
whereas iron def has low ferritin and erythrocytes and high RDW

Beta thalassemia only one with +electrophoresis
Elevated hemoglobin A2 and fetal hemoglobin

91
Q

tx of beta thalassemia

A

β-Thalassemia minor requires no treatment.
• β-Thalassemia major requires early-onset, lifelong transfusion therapy.
• Iron chelation therapy may be indicated if serum ferritin
concentrations exceed 1000 ng/mL.
• Allogeneic HSCT is indicated for severe β-thalassemia major.

92
Q 
what do the following test mean in bleeding disorders 
- the PT and aPTT
• A mixing study 
• Bleeding time
• Thrombin time
• Fibrinogen
A

PT/aptt- monitor for factor deficiencies and factor inhibitors.

Mixing study- differentiates factor deficiency from factor inhibitor by mixing patient plasma with normal plasma and retesting the PT and aPTT.

bleeding time- identifies platelet disorders and vessel-wall integrity; the commercially available Platelet Function

thrombin time- tests the conversion of fibrinogen to fibrin.

fibrinogen- fibrinogen degradation products, and D-dimer are used to identify excessive fibrinolysis.

93
Q

bleeding disorder in liver disease

A

elevated PT/aptt because decrease factor production

  • can still clot because decrease protein C and S
  • fibrinogen low
  • Bleeding may require platelets

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