Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Boards Basic > hematology > Flashcards

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what causes PNH

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

signs of PNH

A
  • chronic hemolytic anemia
  • iron deficiency through urinary losses
  • venous thrombosis (including Budd-Chiari syndrome)
  • pancytopenia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what is pure red cell aplasia

A

absence or a marked decrease of erythrocyte production with

normal leukocyte and platelet counts.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

causes of red cell aplasia

A

T cell autoimmunity (pregnancy, thymoma, malignancy) or

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what will MDS lead to

A

acute leukemia syndrome or death

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

bone marrow finding in mds

A

hypercellular with dysplastic erythroid precursors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

treatment for -5q syndrome

A

lenalidomide

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

CML translocation

A

9:22 chromosome Philadelphia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

what are patients with CML susceptible to

A

transitioning to AML- blast crisis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

how do you know if patient is in blast phase

A

> 20% of leukocytes are blast

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

what testing needs to be done with tyrosine kinase inh

A

ekg because prolonged qt

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
if patient were to have symptoms of essential thrombocytemia what would they be
* vasomotor disturbances such as erythromelalgia (red and painful hands or feet with warmth and swelling) * livedo reticularis * headache * vision symptoms * arterial or venous thromboses
26
diagnosis of ET
splenomegaly and Jak 2 mutation (50%)
27
tx of ET
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.
28
most common causes of thrombocythemia
iron deficiency and infection
29
how to differentiate primary vs secondary polycythemia vera
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.
30
secondary polycythemia vera
hypoxemia (most common), volume contraction because of diuretics, use of androgens, and secretion of erythropoietin by kidney or liver carcinoma.
31
symptoms of polycythemia vera
thrombosis or bleeding, facial plethora, erythromelalgia, pruritus exacerbated by bathing in hot water, and splenomegaly.
32
dx of polycythemia
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.
33
tx for polycythemia vera
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.
34
Hepatic vein thrombosis (the Budd-Chiari syndrome) or portal vein thrombosis should prompt evaluation for
PV
35
cut off for hypereosinophic syndrome
eosinophil >1500, infiltrates in the liver, spleen, heart, and lymph nodes; and systemic symptoms
36
causes of eosinophilia (5)
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
37
clinical features of ALL
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
38
tx for ALL
``` 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
signs of AML
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
diagnosis of aml
bone marrow aspiration and biopsy showing >20% myeloblasts.
41
what is apl
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
tx for APL
all-trans retonoic acids
43
what are patient who take ATRA at risk for and how does it present and what is the tx
g differentiation syndrome. Characteristic findings are fever, pulmonary infiltrates, hypoxemia, and, occasionally, hyperleukocytosis. Treatment is dexamethasone.
44
when to do plt transfusion and leukophoresis in AML
plt transfusion if plt <10k | leukophoreiss >50k
45
normocytic anemia causes based on reticulocyte
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
``` iron deficiency anemia serum iron ferritin TIBC transferrin sat % ```
Serum iron Low Ferritin Low TIBC High Transferrin saturation Low (<10%)
47
``` Inflammatory anemia serum iron ferritin TIBC transferrin saturation ```
Serum iron Low Ferritin High TIBC Low Transferrin saturation Low/Normal
48
most common cause of microcytic anemia
menstrual or GI blood loss or malabsorption | syndromes (celiac disease). Other causes include inflammatory disorders and lead intoxication
49
childhood microscopic iron deficiency should be evaluated for
thalassemia trait, other hemoglobinopathies (thalassemia), or ineffective erythropoiesis (hereditary sideroblastic anemia).
50
signs of iron deficiency anemia
restless legs syndrome, hair loss, and spoon nails
51
causes of macrocytic anemia
* 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
testing for macrocytic anemia and what to do if inconclusive results
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
tx for b12 deficiency
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
tx for folate deficency
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
classic findings in hemolytic anemia
anemia, splenomegaly, elevated reticulocyte count, elevated LDH and indirect bilirubin, decreased haptoglobin, and elevated MCV
56
examples of congenital hemolytic anemia
sickle cell disorders of the erythrocyte membrane (hereditar spherocytosis), enzyme defects (glucose-6-phosphate dehydrogenase deficiency), and thalassemia syndromes.
57
examples of acquired hemolytic anemia
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
Schistocytes and thrombocytopenia
TTP-HUS, DIC, HELLP
59
Schistocytes in a patient with a prosthetic heart valve
Valve leak
60
Erythrocyte agglutination
Cold agglutinin hemolysis (Mycoplasma infection, lymphoproliferative diseases, CLL)
61
Spherocytosis
Autoimmune hemolytic anemia or hereditary spherocytosis
62
Target cells
Thalassemia, other hemoglobinopathy, or liver disease
63
Bite cells
``` G6PD deficiency (suggested by eccentrically located hemoglobin confined to one side of the cell) ```
64
Warm autoimmune hemolytic anemia tx
initial therapy is glucocorticoids. Alternative agents are available for patients unresponsive to glucocorticoids or splenectomy.
65
Cold agglutinin disease tx
primary therapy is cold avoidance or rituximab for persistent symptoms; glucocorticoids or splenectomy are usually ineffective
66
ttp tx
emergent plasma exchange.
67
Hereditary spherocytosis and transfusion-dependent thalassemias tx
splenectomy
68
Severe thalassemia tx
HSCT is standard therapy.
69
Severe PNH tx
eculizumab or HSCT
70
A personal or family history of anemia, jaundice, splenomegaly, or gallstones suggests
hereditary spherocytosis
71
vaccines needed for sickle cell and hemolytic anemia dz
pneumococcal (both 23- and 13-valent), Haemophilus | influenzae type B, influenza, and meningococcal vaccinations.
72
signs of an aplastic crisis
hemoglobin drops >2 - caused by parvovirus b19 or other cytoxic drugs - reticulocyte count low
73
Sickle and Chronic pain involving hips and shoulders
Osteonecrosis (avascular necrosis)
74
sickle and CVAs
Ischemic infarction in children and hemorrhage in adults
75
sickle and Infection with encapsulated organisms
Functional asplenia
76
Sickle cell and Liver disease think
Viral hepatitis, iron overload from transfusions, or ischemic-induced hepatic crisis
77
sickle cell and impotence think
Prolonged or repeated episodes of priapism
78
Sickle cell and proteinuria or isothenuria think
ckd
79
sickle cell and decreased visual acuity
retinopathy
80
initial management of vaso-occlusive disease
hydration, supplemental oxygen for hypoxemia, treatment of any precipitating event, and opioids.
81
tx options for sickle cell
• 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
transfusion threshold for sickle cell
The transfusion target is hemoglobin level <10 g/dL (hemoglobin A level >70%). Do not transfuse patients with simple vasoocclusive pain
83
(–α/αα) [single-gene deletion] clinical syndrome and tx
Silent carrier state that is clinically normal tx: None
84
(– –/αα; or –α/–α) [two-gene deletion] clinical syndrome and tx
α-Thalassemia trait; mild microcytic anemia; normal or elevated erythrocyte count; normal hemoglobin electrophoresis Tx: None
85
(– –/–α) [three-gene deletion] clinical syndrome and tx
Hemoglobin H (β4); severe anemia and usually early death Intermittent transfusion
86
(– –/– –) [four-gene deletion] clinical syndrome and tx
Hydrops fetalis; fetal death In utero transfusion
87
pathophys of b thalassemia
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
β-Thalassemia major | (Cooley anemia) characteristics and tx
Two-gene deletion leading to either no production or severely limited production of β-globin Tx Transfusion, iron chelation; consider splenectomy and HSCT
89
β-Thalassemia minor | (β-thalassemia trait) characteristics and tx
A single β-gene leading to reduced β-globin production with no or mild anemia Tx None
90
how to differentiate iron deficiency anemia and thalassemia
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
tx of beta thalassemia
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
``` what do the following test mean in bleeding disorders - the PT and aPTT • A mixing study • Bleeding time • Thrombin time • Fibrinogen ```
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
bleeding disorder in liver disease
elevated PT/aptt because decrease factor production - can still clot because decrease protein C and S - fibrinogen low - Bleeding may require platelets

Boards Basic (10 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions