Hematology Flashcards
1
Q
What is the most common primary childhood CNS tumors?
A
astrocytoma - develop from astrocytes
2
Q
What are the symptoms of astrocytoma?
A
most patients have symptoms consistent wit increased intracranial pressure (morning headaches
-often benign
3
Q
What are the symptoms when the astrocytoma is located in the cerebellum?
A
weakness, tremor, and ataxia
4
Q
What are the symptoms when the astrocytoma is located in the visual pathway?
A
visual loss, proptosis, or nystagmus
5
Q
What are the symptoms when the astrocytoma is located in the spinal cord?
A
pain, weakness, and gait disturbances
6
Q
How is the dx of astrocytoma made?
A
diagnosis is based on MRI and histologic evaluation of biopsy
7
Q
What is the tx of astrocytoma?
A
combination of surgical resection, radial therapy, and chemotherapy
-have the best posterior fossa tumor prognosis, with a 90% five-year survival rate
8
Q
What is the most common malignant posterior fossa tumor in children?
A
medulloblastoma and represents about 20% of all pediatric CNS cancers
9
Q
What are the characteristics of medulloblastoma?
A
- metastasize through CSF
- it has a bimodal peak at age 3 to 4 years and at age 8 to 10 years but can occur throughout childhood
10
Q
What is the presentation with a medulloblastoma?
A
most commonly with vomiting, headache, nausea, visual changes (double vision), and unsteady walking or clumsiness
11
Q
How is the dx of medulloblastoma made?
A
diagnosis is based on MRI and histologic evaluation of biopsy
12
Q
What is the tx of medulloblastoma?
A
combination of surgery, radiation therapy, and chemotherapy
-the long-term survival with treatment is about 70%
13
Q
What is the 3rd most common CNS tumor in children?
A
ependymoma
-after astrocytomas and medulloblastomas, representing 10% of pediatric brain tumors
14
Q
What is the mean age at diagnosis for ependymoma?
A
6 years, about 30% of ependymomas occur in children <3 years
15
Q
What are the initial symptoms of ependymoma?
A
typically related to increased intracranial pressure
- infants may present with developmental delay and irritability
- changes in mood, personality, or concentration may occur
- seizures, balance and gait disturbances, or symptoms of spinal cord compression (back pain, loss of bladder and bowel control) may occur
16
Q
How is the dx of ependymoma made?
A
diagnosis is based on MRI and histologic evaluation of biopsy
17
Q
What is the tx of ependymoma?
A
surgical resection, usually followed by radiation therapy
- sometimes chemotherapy
- the survival rate depends on age and on how much of the tumor can be removed
- total or near total removal: 51 to 80% survival
- less than 90% removal: 0 to 26% survival
18
Q
What is a retinoblastoma?
A
- malignancy of the retina of the eye
- often presents in children less than 3 year of age
- physical exam - leukocoria (absence of red-light reflex)
19
Q
What is the tx of a retinoblastoma?
A
surgical enucleation of the eye, chemotherapy
20
Q
What is hemophilia?
A
a hereditary bleeding disorder caused by a deficiency in one of two blood clotting factors
21
Q
What are the two forms of hemophilia?
A
- factor VIII in hemophilia A which accounts for 80% of all cases
- factor IX in hemophilia B (Christmas disease)
- *Remember: Hemophilia A = “Aight” and B comes after A which is factor NINE
22
Q
What is hemophilia caused by?
A
caused by severe different X-linked recessive gene abnormalities = the gene abnormalities are inherited through the mother and that nearly everyone with hemophilia is male
23
Q
What are the bleeding patterns and consequences of these two types of hemophilia?
A
hemarthrosis, bruising, and bleeding
24
Q
What is the main symptom of hemophilia?
A
excessive bleeding
- the bleeding may be into a joint or muscle, inside the abdomen or head, or from cuts, dental procedures, or surgery
- recurring bleeding into the joints and muscles can lead to crippling deformities
- bleeding can swell the base of the tongue until it blocks the airway, making breathing difficult
- a slight bump on the head can trigger substantial bleeding in the brain or between the brain and the skull, causing brain damage and death
- a child who has hemophilia bruises easily
- even an injection into a muscle can cause bleeding that results in a large bruise and hematoma
25
What does the severity of the bleeding depend on?
depends on how a particular gene abnormality affects the blood clotting activity of factor VIII or IX
26
What are the characteristics of mild hemophilia?
- people whose clotting activity is 5 to 25% of normal have mild hemophilia that may go undiagnosed
- these people may bleed more than expected after surgery, dental extraction, or a severe injury
27
What are the characteristics of moderate hemophilia?
- people whose blood clotting activity is 1 to 5% of normal have moderate hemophilia
- they have few unprovoked bleeding episodes, but surgery or injury may cause uncontrolled and fatal bleeding
28
What are the characteristics of severe hemophilia?
- serious episodes of bleeding occur and recur after a minor injury or for no apparent reason
- in severe hemophilia, the first bleeding episode often occurs during or immediately after delivery
- the infant may develop a collection of blood under the scalp (cephalhematoma) or may bleed excessively during circumcision
- in serve or moderate hemophilia, a bleeding episode generally occurs before 18 months of age and may follow a minor injury
29
How is hemophilia dx?
increase PTT, normal PT, and platelets, with decreased Factor VIII or IX on assay
- increased PTT and normal platelet count and function
- corrected with mixing studies = indicates a factor deficiency
- if PTT does not correct with mixing studies indicates lupus anticoagulant or factor inhibitor
- normal PT
- normal bleeding time
- most specific test: functional assay for factor VIII (Hemophilia A) or IX (Hemophilia B) to confirm the diagnosis of hemophilia and determine its type and severity
30
What is the treatment of Hemophilia?
treatment often involves the replacement of factor VIII or IX
- people who have hemophilia should avoid situation that might provoke bleeding and should avoid drugs (for example, aspirin and probably also NSAIDs) that interfere with function of platelets
- they should be conscientious about dental care so that they will not need to have teeth extracted
31
What is the most common environmental illness of children in the United States?
lead poisoning
32
What are the characteristics of lead poisoning?
- in children, most organs are susceptible to lead poisoning, especially the brain
- therefore, lead poisoning must remain high on the differential list for pediatric patients
33
What is the most significant source of lead toxicity in children?
environmental exposure
- including exposure to lead-based paint, food, water, and soil
- classic case of lead poisoning involve children inside an old house with paint chips
34
What are the symptoms of lead toxicity in children?
non-specific
- behavioral changes, temperamental lability, irritability, hyper/hypoactivity, developmental delays, abdominal pain, vomiting, constipation, lethargy, headache, ataxia, and seizures
- lead poisoning can also cause anemia
35
How is lead poisoning dx?
once lead poisoning is suspected, whole blood lead levels need to be obtained
- a level of 10 ug/dl is considered positive
- increased serum blood lead level
- basophillic stippling
- decreased or normal MCV
- decreased mean MCH
- hemolysis
- increased indirect bilirubin, LDH
- decreased haptoglobin
36
What is the tx of lead poisoning?
treatment includes preventing further exposure, chelation therapy, and dietary measures
- dimercaprol, CaNaEDTA, penicillamine, and succorer are all agents that can be used to treat lead toxicity
- typically chelation therapy is only indicated if a patient has a blood lead level of ~45 ug/dL
- patients are hospitalized when the levels are about 70 ug/dL
- contact with local health department is only necessary when patients have blood level of greater than 20 ug/dL or if after 3 months the level remain elevated
- siblings and other children who live in the household or attend the same school or daycare should be tested
37
What accounts for the greatest percentage of cases of childhood malignancies?
leukemia
- there are 3,000 new cases of leukemia each year in the US
- acute leukemia constitutes 97% of all childhood leukemias and is subdivide into acute lymphocytic leukemia (ALL) and acute nonlymphocytic anemia, also known as acute myelogenous leukemia (AML)
- chronic leukemias make up 3% of childhood leukemias
38
What are characteristics of Acute lymphocytic leukemia?
- CHILD + Lymphadenopathy + bone pain + bleeding + fever in a CHILD, bone marrow >20% blasts in bone marrow
- the most common pediatric neoplasm accounts for 80% of all cases of childhood acute leukemia
- most common childhood malignancy peak age 3-7 y/o
- highly responsive to chemotherapy (remission >90%)
- ANC < 1000
39
What are the characteristics of acute myelogenous leukemia?
- AML accounts for 20% of all cases of childhood leukemia
- far more common in males than females
- patients with AML may develop a soft-tissue tumor called a chroma in the spinal cord or skin
- the lesion shave a greenish hue
- smear Auer rods
40
What are the sx of leukemia?
- symptoms usually develop less than 4 weeks before diagnosis
- initial symptoms include lethargy, malaise, and anorexia
- 25% of children develop bone pain or arthralgia caused by leukemic infiltration of the perichondral bone or leukemic expansion of marrow cavity
- progressive bone marrow failure leads to pallor, ecchymoses or petechiae (50%) and fever (25%)
41
What are the ddx of leukemia?
aplastic anemia, ITP, epstein-barr, and other malignancies
42
How is leukemia dx?
- anemia and thrombocytopenia are present at diagnosis in 90% of cases
- the anemia is normochromic and normocytic
- decreased marrow production of red blood cells leads to low reticulocyte count
- WBC count is low <5,000/mm3 in one-thirds of patients, normal in one-third, and high >20,000 mm3 in one-third
- blast cells are frequently seen on peripheral smear especially if WBC is normal or high
- bone marrow examination FNA and biopsy confirms DX
43
What is the tx of ALL?
chemotherapy
- highly responsive to combination chemotherapy with remission of >90%
- stem cell transplant if relapse
44
What is the tx of AML?
combination chemotherapy + bone marrow transplant
-tumor lysis syndrome - lethal side effect to chemotherapy initiation - treat with allopurinol and manage acute renal failure
45
What is Hodgkin lymphoma?
a tumor derived from lymphocytes - specifically B-cells which mainly reside in lymph nodes
46
What are the characteristics of Hodgkin lymphoma?
- bimodal age distribution (15-35) and (>60)
- painless cervical or supraclavicular lymphadenopathy
- upper body lymph nodes: neck, axilla, shoulder, chest (mediastinum)
- fatigue, wt loss, fever, chills, and drenching night sweats for > 1 month
- B symptoms are common = weight loss >10%, T >38 C, night sweats
- associated with EBV (40% of patients)
- spreads contiguously (nearby) = rarely extra nodal
- two types: Classical (4 types) and nodular lymphocytic
- classical: nodular sclerosis, mixed cellularity, lymphocyte-rich, lymphocyte depleted
47
How is Hodgkin lymphoma dx?
if the diagnosis is suspected, a chest radiograph should be obtained to search for mediastinal adenopathy
- diagnose with excisional biopsy of lymph node, may need bone marrow
- CBC normal, elevated ESR
- CXR - mediastinal mass (adenopathy)
- CT scan to establish the stage
- Excisional biopsy of the lymph node shows Reed-Sternberg cells
- Reed-Sternberg cells are pathognomonic - B cells fused together forming a large cell with two nuclei "owl eyes"
48
What is the tx of Hodgkin lymphoma?
depends on stage, age, and general health
- chemotherapy, radiation therapy - is highly curable compared to non-hodgkin lymphoma
- prognosis = excellent 5-year cure rate (60%)
49
What is Non-hodgkin lymphoma?
a tumor derived from lymphocytes - specially from B-cells and T-cells
50
What are the symptoms of non-hodgkin lymphoma?
- painless lymphadenopathy
- release of cytokines causes fever, drenching nigh sweats, and weight loss
- extranodal involvement
- GI tract = bowel obstruction
- bone marrow = fatigue, easy bruising, recurrent infection
- spinal cord = loss of sensation
51
What are the two types of Non-hodgkins lymphoma?
B cell and T cell lymphoma
52
What are the characteristics of Non-hodgkins B cell lymphoma?
- diffuse large B cell lymphoma = most common and agressive
- follicular lymphoma = indolent, from chromosomal translation
- Burkitt lymphoma = high aggressive, starry sky appearance on microscopy
- mantle cell lymphoma = aggressive
- marginal zone lymphoma = indolent
- nodal marginal zone
- splenic marginal zone
- lymphoplasmacytic lymphoma = indolent
53
What are the characteristics of non-hodgkins T cell lymphoma?
- adult T-cell lymphoma = sometimes referred to as a leukemia
- mycosis fungoides = T cell lymphoma of skin
54
What is the dx of non-hodgkins lymphoma?
usually NO Reed-Sternbery cells
- imagin studies = CT scan to help establish the stage based on extent of nodal and extra nodal involvement
- lymph node biopsy
55
What is the tx of non-hodgkins lymphoma?
chemotherapy and radiation therapy
- depends on subtype, aggressiveness and spreading
- worse prognosis than Hodgkin lymphoma
56
What is neutropenia?
ANC < 1000
- fever is the earlier and only sign
- hx of chemotherapy treatment
57
What is the dx of neutropenia?
- CBC, CMP, blood cultures, UA
| - CXR, CT/US, LP
58
What is the tx of neutropenia?
- outpatient (low risk): Cipro + augmentin; moxofloxacin
- monotherapy *pseudomonas; cefepime, imipenem, augmentin
- dual therapy: *unstable mono therapy + Vancouvers or flagyl (abd)
59
How is anemia defined as?
low hematocrit and/or hemoglobin relative to normal values
60
What are the symptoms of anemia?
weakness, fatigue, dyspnea on exertion
61
What is the physical exam findings of anemia?
pallor, tachycardia, systolic flow murmur, jaundice, positive stool guaiac
-may present as angina
62
What anemia are microcytic (MCV, 80 fl)
- iron deficiency
- anemia of chronic disease
- thalassemias
- sideroblastic anemias
- lead poisoning
63
What are the two types of normocytic (MCV 80-100 fl)?
hemolytics and non-hemolytic
64
What are the hemolytic anemias?
```
Intrinsic
-hereditary spherocytosis
-G6PD deficiency
-pyruvate kinase deficiency
-sickle cell anemia
-HbC disease
Extrinsic
-autoimmune anemia
```
65
What are the non-hemolytic anemias?
- iron deficiency
- anemia of chronic disease
- chronic kidney disease
- aplastic anemia
66
What are the two types of macrocytic (MCV >100 fl)?
megaloblastic (immature cells) and non-megaloblastic
67
What are the megaloblastic (immature cells)?
- folate deficiency
- B12 deficiency
- copper deficiency
- drug-induced
68
What are the non-megaloblastic anemias?
- alcohol use disorder
| - liver disease
69
What does microcytic follow with?
- ferritin
- iron
- TIBC
70
What does normocytic follow with?
- coombs test
- iron studies
- bilirubin
71
What does macrocytic follow with?
- RBC folate
| - serum B12
72
What are the lab findings for iron deficiency?
low ferritin, low serum Fe, high TIBC
73
What are the lab findings for thalassemia?
HbF increased, low MCV, hemolysis
74
What are the lab findings for sideroblastic?
high ferritin, high serum Fe, low TIBC
75
What are the lab findings for lead?
low MCV, basophilic stippling, increased free erythrocyte porphyrins
76
What are the lab findings of hemorrhage?
watch CVP, not the Hct
77
What are the lab findings of chronic disease/renal failure?
high ferritin, low serum FE, low TIBC
78
What are the lab findings of infection?
osteomyelitis, HIV, mycoplasma, or EBV
79
What are the lab findings of bone marrow disease?
leukemia, lymphoma, or myelodysplasia
80
What are the lab findings of hemolytic anemia?
-mechanical = increased reticulocyte count
-immunologic = positve coombs test
Intrinsic hemolysis
-GEPD/Herediatry spherocytosis = decreased serum haptoglobin
Extrinsic hemolysis
-cold agglutins = osteomyelitis, HIV, mycoplasma, or EBV
-autoimmune = increased LDH, K+ and bilirubins, decreased heptoglobin
-mechanical destruction = microangiopathic hemolytic (TTP, HUS, or DIC)
81
What are the lab findings of B12 and Folate deficiency?
increased MCV, increased LDH, decreased haptoglobin
82
What are the lab findings of drug exposure?
history of phenytoin, phenobarbital, or MTX
83
What is primary hemostasis?
formation of a weak platelet plug and causes mucocutaneous bleeding
84
What are the primary hemostasis problems?
usually cause petechiae (pinpoint superficial skin bleeds), anterior epistaxis (mild nosebleeds), immediate bleeding after surgical procedures, like tooth extraction, or bleeding from mucosal surfaces, like gingival, gastrointestinal, or vaginal bleeding
85
What is secondary hemostasis?
problems with coagulation factors and causes deep tissue bleeding
86
What are the secondary hemostasis problems?
can develop large bruises (ecchymoses), deep tissue hematomas or hemarthrosis (bleeding inside the joint space), posterior epistasis (severe nosebleeds), persistent bleeding after surgical procedures, as well as intracerebral hemorrhage
87
What might thrombocytopenia caused by?
decreased production, increased destruction or sequestration
88
What are the bleeding disorders of decrease in platelet function
Von Willebrand Disease
- type 1: decreased quantity of VwF
- type 2: decreased function of VwF
- Bernard-Souiler disease
- Glanzmann thrombasthenia
- Uremic platelet dysfunction
- Result of medications like aspirin, non-steroidal anti-inflammatory drugs or NSAIDs, and clopidogrel
89
What are the bleeding disorders of decreased clotting factors?
- Anticoagulant medications: heparin, warfarin
- Hemophilia
- Liver cirrhosis
- Vitamin K deficiency
- DIC
90
What do patients with primary hemostatic have problems with?
a prolonged bleeding time
91
What are the characteristics of prolonged prothrombin time (PT)?
- extrinsic and common coagulation pathways
| - seen in bleeding from warfarin
92
What are the characteristics of prolonged partial thromboplastin time (PTT)?
- intrinsic and common coagulation pathways
| - seen in bleeding from heparin
93
What might a CBC show for von Willebrand disease?
might show anemia due to the loss of red blood cells and a normal platelet count
- decreased von Willebrand's factor (vWF) and decreased factor VIII
- a PTT may be normal or elevated in patients with decreased factor 8 activity
94
What are the effects of anticoagulant medications on PT and PTT?
heparin, warfarin
- patients bleeding from warfarin have a prolonged PT and normal PT
- patients bleeding from heparin have a prolonged aPTT and normal PT
95
What are the characteristics of hemophilla?
deficiencies in coagulation factors
- Hemophilia A and B are X-linked recessive disorders, so they almost exclusively affect males, and lead to deficiencies in factor 8 and factor 9, respectively
- typically there's a prolonged aPTT, with a normal PT and platelet count
- infant males often develop excessive bleeding from circumcision or can develop a cephalohematoma which is bleeding under the scalp due to trauma from the delivery
- children can also develop a hemarthrosis once they start walking and falling
- repeated episodes of hemarthrosis can cause the joint deform, severely restricting the range of motion and causing chronic pain
96
What are the PT and PTT for liver cirrhosis, vitamin K deficiency, and DIC?
prolonged PT and prolonged PTT
97
What are the lab findings for cirrhosis?
decreases albumin, increased bilirubin, and increased AST and ALT (early), or decreased AST and ALT (late)
98
What infants get a Vitamin K injection?
all infants
99
What is DIC?
overactivation of the coagulation system = increased PT and PTT
-thrombocytopenia, peripheral blood smear showing microangiopathic hemolytic anemia, schistocytes
