Pathoma Neo Blood RBC WBC Flashcards
(134 cards)
1
Q
What problem are you likely to get from a rattlesnake bit? Scorpion sting?
A
Rattlesnake = DIC; Scorpion = acute pancreatitis
1
Q
Person had a laparoscopic surgery and now has multifocal ischemic necrosis of bone?
A
Caisson Disease; a chronic form of gas embolism, from the gas in the pneumoperitoneum
2
Q
What is the general purpose of primary and secondary hemostasis?
A
Primary = platelet plug; Secondary = stabilize the platelet plug, i.e. fibrin
2
Q
Why does Hairy Cell Leukemia present with splenomegaly?
A
Hairy cells get trapped in the red pulp
2
Q
Which cancer involves mature CD4 positive cells in the skin?
A
Mycosis fungoides
3
Q
What kind of molecule is c-KIT? What cancer is it implicated in?
A
Growth factor receptor; Gastrointestinal Stromal Tumor (interstitial cells of Cajal)
3
Q
Which forms of autoimmune hemolytic anemia cause intra and extravascular hemolysis?
A
IgG (warm) causes extravascular; IgM (cold) causes intravascular
4
Q
What is the genetic makeup of HBV and HCV?
A
HBV = DNA, HCV = RNA
4
Q
What would kill a person deficient in Cystathione Beta Synthase?
A
Thrombosis
4
Q
What anatomic locations require prophylaxis with B-ALL?
A
Testes and CSF
4
Q
What are crystal aggregates of myeloperoxidase referred to as?
A
Auer rods
5
Q
What is producing the antiboides in ITP?
A
Splenic plasma cells (make IgG against GPIIb/IIIa); The splenic MO then eat the platelets = thrombocytopenia
5
Q
Why to OCP’s increase risk of thrombosis?
A
Estrogen increases production of coagulation factors
6
Q
What is the most common cause of thrombotic thrombocytopenic purpura?
A
autoantibody to ADAMTS13
7
Q
Where in the kidney are RBC’s most likely to sickle?
A
Renal medulla because it is hypoxic and hypertonic
8
Q
What does chromogranin stain for?
A
Neuroendocrine cells
8
Q
What is the most common cause of thrombocytopenia in both children and adults?
A
ITP
8
Q
How can you differentiate DIC from a disorder of fibrinolysis?
A
DIC will have decreased platelet count and D-dimer? Fibrinolysis does not have fibrin split products and there is no consumptive platelet issue
8
Q
Regarding infection with EBV (heterophile Ab positive IM), generalized LAD is from hyperplasia of what?
A
paracortex of LNs
9
Q
Which cancers are N-MYC and L-MYC implicated in?
A
N-myc = neuroblastoma (adrenal medulla, kid, no HTN); L-MYC = lung carcinoma
9
Q
Who is G6PD the most severe in, Africans or Mediterraneans?
A
Mediterranian variant is the worst
9
Q
First line Tx of CML
A
imatinib
10
Q
What is a major way that tumor cells can evade “immune surveillance”?
A
downregulating MHC I
11
Q
What is the main role of FGF in the body?
A
angiogenesis (although it seems like it should be collagen synthesis)
11
Which vitamin functions as a cofactor in protoporphyrin synthesis?
B6 (water soluble)
11
Why do splenic macrophages eat spherocytes in hereditary spherocytosis?
Because they don?t navigate the sinusoids as well due to their large bulky shape (Tx: splenectomy)
13
Why do you have to correct the reticulocyte count in cases of anemia?
Because there is a decrease in total RBC so the retic count is falsely elevated
14
A patient with follicular lymphoma whose lymph node is now enlarging may have\_\_\_\_\_\_\_\_\_\_
transformed to diffuse large B-cell lymphoma
15
What lab test is elevated in Hemophilia A and B?
PTT as factors VIII and IX are both part of the intrinsic pathway
17
What is the function of haptoglobin? When is its value decreased?
To bind to hemoglobin in the blood, decreased in cases of intravascular hemolysis because it is binding up all of the hemoglobin
17
What is the most common cause of death in children with Sickle Cell?
HiB because of autosplenectomy
17
Splenomegaly in mono is due to hyperplasia of Downey lymphocytes where?
in PALS (within the adventitia of splenic arteries)
18
How can you use the presence of tingible body macrophages to differentiate between follicular hyperplasia and follicular lymphoma?
there are no tingible body macrophages in follicular lymphoma
18
What receptor is acted on by malignant plasma cells in multiple myeloma that leads to hypercalcemia?
RANK receptor on osteoclasts
20
Describe the basic biochemistry of a mutated Ras protein
Ras is a G protein that should cleave GTP to GDP to make it inactive. Mutated Ras basically has no "off switch" so GTP stays on.
21
What is eosinophilic granuloma?
Benign proliferation of langerhans cells in bone that presents as a pathologic fracture
22
What is a metabisulfite screen?
Causes cells with any amount of HbS to sickle, useful in sickle cell anemia
23
Why do obstetric complications cause DIC?
Tissue thromboplastin in the amniotic fluid causes DIC
25
Regarding platelet disorders, what is the most common type of mucosal bleed?
Epistaxis (also keep Osler-Weber Rendu in mind if there are bleeds elsewhere)
27
What are two ways in which hepcidin (anemia of chronic disease) sequesters Fe?
limits iron transfer from macrophages to erythroid precursors; decreases EPO production
28
What is the characteristic finding of HbC disease on blood smear?
HbC crystal
29
Explain why issues with protoporphyrin synthesis lead to ringed sideroblasts
The ferrochetalase reaction occurs in the mitochondria? Here, protoporphyrin is added to Fe to form Heme. When protoporphyrin is defective iron accumulates in the mitochondria forming ringed sideroblasts
30
Reed-Sternberg cells are classically positive for what CD markers?
CD15 and CD30
31
What is the most common leukemia overall?
Chronic lymphocytic leukemia
32
What is the glutamic acid to lysine mutation on B globin?
HbC disease (typified by HbC crystal on blood smear)
33
Discuss the regulation of the Retinoblastoma tumor suppressor gene
Rb holds the E2F transcription factor. When the cyclinD/CDK4 complex phosphorylates Rb it releases E2F allowing for progression of G1 to S
33
What is the best test for immune hemolytic anemias?
Direct Coombs
34
Hemoglobin is made of heme and globin. The heme is made of Fe and Protoporphyrin IX. What is called if there isnt enough Fe? Enough Protoporphyrin?
Fe = Iron Deficiency Anemia (or in some cases, anemia of chronic disease); Protoporphyrin = Sideroblastic anemia
36
What does the term diffuse mean in diffuse large B cell lymphoma?
You cannot identifiy a mantle, follicle , marginal zone, etc.
37
What is the main cause of death in paroxysmal nocturnal hemoglobinuria? Why?
Thromboses i.e. Budd Chiari and cerebral vein thrombosis? Because damage to platelets releases enzymes that activate more platelets and cause thrombosis
38
What kind of molecule is TdT? (Used to identify acute lymphoblastic leukemia)
DNA polymerase
39
What is the earliest sign of whole body radiation?
lymphopenia
40
What cluster of differentiation marker identifies a hematopoietic stem cell
CD34
41
What will you see on spiral CT of a person with pulmonary embolus?
vascular filling defect
42
What is the screening test for paroxysmal nocturnal hemoglobinuria and the confirmatory test?
Sucrose test; acidified serum test
43
Which aspects of platelet plug formation are impaired in uremia?
impaired platelet adhesion and aggregation
43
Explain how gastrectomy predisposes to both Fe def and B12 anemia
Acid helps maintain Fe2 which is desirable over Fe3? Loss of stomach means loss of parietal cells, no IF = no B12
44
Describe a Sezary cell
Ceribriform nuclei
45
What region is characteristically involved in acute monocytic leukemia?
infiltration of the gums
47
In which cancer is basophilia classically seen?
Chronic myelogenous leukemia
48
What does vimentin stain for?
Mesenchymal tissue (mesoderm, bone etc.)
50
Which kidney cells produce EPO?
peritubular interstitial cells
51
What feature do many leukemic blast cells have in their nucleoli?
The nucleoli are often punched out
52
Differentiate among DMT-1, Ferroportin, and transferrin.
DMT-1 takes heme from the intestine and into the enterocyte (DMT-1); Ferroportin takes heme from the enterocyte to the blood (basolateral); Transferrin takes iron through the blood to liver and bone marrow for storage in MO as ferritin
53
What does desmin stain for?
Muscle
54
What is Hand-Schuler-Christian disease?
Malignant proliferation of Langerhans cells characterized by scalp rash, lytic skull defects, diabetes insipidus, and exophthalmos in a kid
56
Explain the relationship between p53 and BAX
p53 upregulaes BAX which disrupts bcl-2? Thus, if DNA damage is beyond repair, p53 is expressed, upregulates BAX, BAX disrupts the anti-apoptotic bcl-2 which leads to apoptosis by allowing cytochrome c to leak out
56
Why is there an increased risk of gout in polycythemia vera but not in essential thrombocythemia?
Because in PV you are making too many RBC's in the process, the nucleus must be extruded = purines = gout. In ET, it is just too many platelets, they have no nuclei.
57
Name 4 small B cell lymphomas; 1 intermediate; 1 large
Small B: follicular lymphoma, Small lymphocytic lymphoma, mantle cell lymphoma, marginal zone lymphoma? Intermediate = Burkitt's; Large = diffuse large B
58
How can you use the reticulocyte count to differentiate the two broad causes of normocytic anemia?
Low retics = underproduction anemia; High retic = destructive anemia
60
When B12 donates the methyl group given to it by folate, it donates it to homocysteine to form \_\_\_\_\_\_\_
methionine
62
PNH predisposes to what cancer?
AML since the mutation in the GPI anchor is ultimately a myeloid line mutation.
63
Where doe the term mononucleosis come from?
Atypical (Downey) lymphocytes (CD8 positive) look like monocytes
65
Which disease would lead to heparin being useless?
ATIII deficiency (as in nephrotic syndromes) ;you would have to give coumadin to these pts to stop them from being in a hypercoagulated state
66
Which growth factor induces cellular growth in some astrocytomas?
PDGFB
67
Why would Whipple's Disease lead to easy bruising?
Malabsorption syndrome would decrease absorption of ADEK and K is responsible for gamma carboxylation of II, VII, IX, X, and C/S
67
Discuss the treatment of Hairy cell leukemia
Tx = Cladribine which is an adenosine deaminase inhibitor; basically induces ADA deficiency
69
Extravascular hemolysis may lead to gallstones of what color?
Black bilirubin gallstones
71
When will you see follicular hyperplasia?
HIV and Rheumatoid arthritis
72
Why does sepsis cause DIC?
LPS causes IL-1 and IL-6 production which causes endothelial cells to make tissue factor
73
Why does PNH attack at night?
The shallow breathing of sleep yields a respiratory acidosis that activates complement. The mutated GPI anchor means that DAF (decay accelerating factor) cannot shut off and they get lysis of RBCs and hemoglobin in urine and hemosiderin in tubular cells
73
What makes up the bulk of the mass of Hodgkin lymphoma?
Reactive cells against Reed-Sternberg cells
74
In Hodgkin lymphoma, what is a lacunar cell?
A reed sternberg cell, they are often present in lake like spaces
75
Explain why thalassemia is a microcytic anemia but sickle cell anemia is a hemolytic anemia
Both have mutations in globin chains. Thalassemia leads to underproduction of globin which leads to low Hb = anemia; SS anemia results in abnormal globin that cause the cells to sickle and hemolyze = hemolytic anemia
77
What are 3 ways to differentiate CML from a leukemoid reaction?
1 )Negative Leukocyte Alkaline Phosphatase; BASOPHILIA; t(9;22)
78
What are 4 general causes of microcytic anemias?
Iron deficiency anemia, Anemia of chronic disease, Sideroblastic anemia, Thalassemia
80
What can be given to treat leukopenia?
GM-CSF or G-CSF
81
What is a normal bleeding time?
2-7 minutes
82
Where can you find ADP in a platelet?
Dense granules? Released upon binding to vWF on endothelium and leads to upregulation of GpIIb/IIIa receptor for secondary hemostasis
84
How do you differentiate an acquired Factor VIII inhibitor (antibody) from a true hemophilia A patient?
mixing study? In hemophilia A, PTT would correct; in coagulation factor inhibitor PTT would not correct
85
Germline mutations in the Retinoblastoma gene predispose to what 2 cancers?
Retinoblastoma and Osteosarcoma
86
A person has been chronically on antibiotics and now finds that they bruise easily? Dafuq?
Chronic antibiotics can kill of gut flora leading to vitamin K deficiency = no gamma carboxylation of II, VII, IX, and X and C/S
88
Why would a high cortisol state (initially) cause leukocytosis?
Causes WBCs to separate from the pulmonary vasculature
90
What is a heterophile antibody?
IgM antibody that reacts with horse or sheep RBC
91
What drug may cause a sideroblastic anemia?
Isoniazid since it depletes B6. B6 is used in the first reaction by ALAS
92
What is the significance of CD16?
It may be seen in the blood when an immature cell (leukocyte) is released prematurely into the blood as in a leukocytosis (i.e. an infection)? IT INDICATES A DECREASED NUMBER OF Fc RECEPTORS
93
What are 4 myeloproliferative disorders?
These are MATURE myeloid cancers: Chronic myelogenous leukemia, Polycythemia vera, Essential thrombocythemia, and Myelofibrosis
95
What genotype is associated with ALL?
Trisomy 21
96
Which Langerhans cell Histiocytosis can present with diabetes insipidus and exophtalmos?
Hand-Schuler-Christian disease
98
If petechiae are from a hemostatic disorder what is probably the issue?
platelets (thrombocytopenia); not typically seen in coagulation cascade issues
100
What is prothrombin 20210A?
point mutation that leads to increased expression of prothrombin = hypercoagulable state
101
What is the TIBC actually measuring?
The amount of transferrin (i.e. if this is high then ferritin will be low)
101
Differentiate a sezary cell from a HTLV-1 affected cell
Ceribriform nuclei; cloverleaf nuclei
102
Which translocations in B-ALL are associated with good and bad prognoses?
GOOD: t(12;21)? BAD t(9;22) i.e. the Philadelphia chromosome in a kid IS BAD NEWS!!!
103
Discuss the ristocetin assay. What does an abnormal test indicate?
Ristocetin causes platelet GpIb to bind to vWF leading to agglutination. Lack of vWF as in von Willebrand Disease = abnormal ristocetin assay
104
Name the 4 hematogenous spreading carcinomas
Renal Cell Carcinoma (renal vein); Hepatocellular carcinoma (hepatic vein; budd chiari, no JVD), Follicular carcinoma of the thyroid, and Choriocarcinoma
105
Which chemotherapeutic agent may lead to megaloblastic anemia?
5-FU, methotrexate etc.
106
Where does ferrochetalase attache iron to protoporphyrin to from heme?
mitochondria
107
Mutations for alpha thalassemia occur on which chromosome?
16
108
Using tDt and myeloperoxidase (MPO) would distinguish what?
tDt = lymphoblasts, it is not present in mature lymphocytes; MPO = myeloblasts
109
What is the deal with Heparin Induced Thrombocytopenia? What is the deal with warfarin skin necrosis?
HIT = antibodies to heparin/platelet factor 4 complex? The rupturing of platelets leads to activation of other platelets and thrombosis; Warfarin skin necrosis occurs in protein C deficient pts who enter a transient hypercoagulable state
111
A B cell may express its light chains using which 2 proteins?
Kappa or Lambda, more commonly kappa
112
What lab values are elevated in von Willebrand's disease?
both bleeding time and PTT (mildly) because vWF protects factor VIII
114
Definitive test for EBV
Serological test for EBV capsid antigen
115
What kind of lymphoma is a gastric MALToma? It may regress with Tx of what?
Marginal zone lymphoma; H. pylori
116
What 2 bugs can cause Fe deficiency anemia? What causes B12 def anemia?
Ancylostoma duodenale, Necator americanus; Diphyllobothrium latum
117
The transformation of chronic lymphocytic leukemia to a diffuse large B cell lymphoma is called \_\_\_\_\_\_\_\_\_
Richter transformation
118
A cell with a decreased number of Fc receptors is identified with \_\_\_\_\_\_
CD16
119
vWF is from the Weibel-Palade bodies of the endothelium (along with P-selectin) and also from the _______ granules of the platelet
alpha granules of platelet contain von Willebrand factor
120
What does thrombomodulin do?
Redirects thrombin to activate protein C
121
What is Letterer Siwe Disease
Langerhans histiocytosis that presents with rash and cystic skeletal defects
122
Which factors get activated by 1) Tissue thromboplastin 2) Subendothelial collagen
1) Factor VII (extrinsic) 2) Factor XII (intrinsic)
123
What does naphthylamine cuase? Where can you find it?
urothelial carcinoma of the bladder (in cigarrettes)
124
What disease is diagnosed with an osmotic fragility test?
Hereditary spherocytosis
125
What symptoms are different in HUS and TTP?
HUS more commonly has kidney involvement and TTP more commonly has CNS involvement
126
What is the general cause of a microcytic anemia?
Decreased production of hemoglobin with excess divisions leading to microcytosis. That is the bone marrow is attempting to correct the problem by making more RBCs but they are smaller
127
Which cancer may be associated with eosinophilia?
Hodgkin lymphoma
128
What is a macroovalocyte?
A large RBC seen in megaloblastic anemia
129
What is the leading cause of death in children?
accidents
130
What does it mean if there is a high Free Erythrocyte Protoporphyrin?
That there is a lot of protoporphyrin that is not bound to Iron, in other words there is Iron deficiency anemia
131
Disease with decreased ADAMTS13
Thrombotic Thrombocytopenic purpura (schistocytes = helmet cells)
132
What does a D-Dimer tell you about fibrinogen?
NOTHING it only tells you that there are FIBRIN split products
133
What is the most common cause of death in multiple myeloma and why?
infection since the IgG or IgA being produced is crappy and lacks antigen specificity
134
Why is CLL more likely to involve lymph nodes than ALL?
CLL involves more mature lymphocytes, since they are more like what they are actually supposed to be they go to LN, where they truly belong
