Hemonc Flashcards
Heparin to warfarin bridge is necessary because
Proteins c and s have shorter half lives than other factors leading to paradoxical hypercoagulability
For rapid reversal of warfarin give
Ffp or otherwise vit K
For reversal of heparin give
Protamine sulfate
Mech of lmwh (enoxaparin)
Mainly inhibits factor Xa
In hemophilia, pt is __, ptt is ___ and bleeding time is ___
Ptt is prolonged
Pt and bleeding time is normal
What is cryoprecipitate
Mix of factor VIII and fibrinogen more concentrated than ffp
Mild hemophilia can be treated with
Desmopressin
How does desmopressin work to tx hemophilia
Releases factor VIII from endothelial cells
Best initial test to dx hemophilia
Mixing study-mix pt plasma with normal plasma which will correct the ptt
Most accurate test for hemophilia
Obtain specific factor assays for factors VII, VIII, IX, XI and XII
Most accurate dx test for vwd
Decreased agglutination seen on ristocetin cofactor assay
In vwd, what will happen to bleeding time, pt, ptt, and platelet count
Increased bt
Increased ptt (possibly ) due to low factor viii levels
Normal pt and platelet counts
Tx for vwd
Desmopressin
Ocps can be used for menorrhagia
Aspirin ___ risk of bleeding in vwd pts
Increases
Mech of factor V Leiden
Hypercoagulable state
Factor v is resistant to inactivation by protein C
Hypercoagulable state with skin or tissue necrosis following warfarin admin. What am I?
Protein c or s deficiency
Aps is often associated with what two conditions
Sle and rheumatoid arthritis
What tests confirm aps
Lupus anticoagulant and anticardiolipin antibody
Tx of dvt or pe in pts with hypercoagulable state
Heparin followed by 3-6 mos warfarin (longer if it’s a repeated event)
Tx of hit
Discontinue heparin then start argatroban or lepirudin
Dic is characterized by what two things
Thrombosis and hemorrhage
Etiology of dic
Deposition of fibrin in small blood vessels leading to thrombosis and end organ damage; also depletion of clotting factors and platelets leading to bleeding diathesis
Three common associations with dic
Obstetric complications, sepsis and pancreatitis
In dic factor Viii is
Depressed
Three causes of microangiopathic hemolytic anemia
Hus
Ttp
Dic
Etiology of ttp
Deficiency of vwf cleaving enzyme adamts-13 resulting in large vwf multimers that aggregate platelets and create platelet microthrombi. Rbcs are thus fragmented.
Low plt count, microangiopathic hemolytic anemia, neuro changes (delirium, seizure, stroke), impaired renal function, fever. Think
Ttp
Severe increases in creatinine level are more suggestive of hus or ttp
Hus
Platelet transfusion in ttp is
Contraindicated
Tx for ttp
Plasma exchange
Etiology of itp
IgG antibodies formed against platelets
In itp, bone marrow production of platelets is __ and there are __ megakaryocytes in the marrow
Up
Increased
Differential of thrombocytopenia
Hit shoc
Hit or hus Itp Ttp or treatment (meds) Splenomegaly Hereditary (wiskott Aldrich) Other (malignancy) Chemo
Tx of itp if plt count >30,000
None
Tx of itp if plt count <30,000
Corticosteroids or ivig
Thrombocytopenia, Increassd creatinine and schistocytes acutely in a school aged kid, think
Hus
Causes of microcytic anemia
Tails
Thalassemia Anemia of chronic dz Iron def Lead poisoning Sideroblastic anemia
Is tibc high or low in anemia of chronic disease? How about ferritin?
Low and high
Is tibc low or high in iron def anemia? How about ferritin?
Tibc high
Ferritin low
Etiology of anemia of chronic disease
Body hides iron in form of ferritin to limit bacterial proliferation
How does sideroblastic anemia present
Low hemoglobin, high serum iron, basophilic stippling (in lead poisoning) and ringed sideroblasts
Hypersegmented neutrophils are a characteristic finding of
Megaloblastic anemia
Vit b12 def has __ mma and __ homocysteine; folate def has __ mma and __ homocysteine
High and high; normal and high
Hereditary spherocytosis is due to a deficiency in
Spectrin or ankyrin
Tx of spherocytosis
Splenectomy
Why can sickle pts have polyuria or nocturia
Due to sickling happening in inner medulla Of kidneys
Etiology of aplastic anemia
Failure of blood cell production due to destruction of bone marrow cells
Cafe au lait spots, short stature, radial/thumb hypoplasia/aplasia. What am I?
Fanconi anemia
Tx of aplastic anemia
Blood transfusion and stem cell transplantation
How does hydroxyurea work
It stimulates production of fetal hemoglobin
Polycythemia Vera is due to
Clinal proliferation of a pluripotent marrow stem cell due to a jak2 mutation
Epo in pcv
Normal
Tx of pcv
Hydroxyurea and ifn
Telltale sign of porphyria
Pink urine
What happens to reflexes in porphyria
Areflexia
5 ps of acute intermittent porphyria
Painful abdomen, port wine colored urine, polyneuropathy, psych disturbances, precipitated by drugs (cyp450 inducers, alcohol, starvation)
Auer rod think
AML
Most common childhood malignancy
ALL
AML on peripheral smear
Large myeloblasts with round or kidney shaped nuclei
Smudge cells are characteristic of
CLL
Philadelphia chromosome t(9,22) is associated with
CML
Which leukemia tends to present at age <13
ALL
Which leukemia tends to present at age 13-40
AML
Which leukemia tends to present at age 40-60
CML
Which leukemia tends to present at age >60
CLL
What is a leukomoid reaction
Acute inflammatory response to infection with increased neutrophils and a left shift
In cml lap is ___ and in leukomoid reaction lap is __
Low
High
Cll shows ___cytosis, cml shows ____cytosis
Lymphocytosis (elevation of nk, t or B cells)
Granulocytosis
Acute promyelocytic leukemia (apl) a subtype of aml can be treated with
All-trans-retinoic acid
Tx of cml
Imantinib (gleevec)
What is more common non-Hodgkin or Hodgkin lymphoma
NHL
Most nhl cases are _ cell in origin
B
Tx of nhl may be complicated by ___ syndrome, which causes what electrolyte abnormalities
Tumor lysis syndrome in which rapid cell death releases intracellular contents and leads to hyperkalemia, hyperphosphotemia, hyperurecemia And hypocalcemia
Fever, wt loss and night sweats are the _ sx of
B; Hodgkin lymphoma
Crab for mm
Hypercalcemia
Renal involvement
Anemia
Bone lytic lesions/back pain
Hypercalcemia sx
Renal stones Bone and abdo pain Confusion Polyuria Weakness Nausea
Protein:albumin gap is often __ in multiple myeloma
Elevated
Tx of mm in pts <70? >70?
Below 70: bone marrow transplant
Above: melphalan and prednisone
What are dutcher bodies? What do you see them in?
They are pas+ igm deposits around the nucleus seen in waldenstrom macroglobulinemia
Do pts with mm often have renal dysfunction
Yes
Define neutropenia
Anc<1500
Tx of mgus
None But see regularly to prevent progression to mm
Define eosinophilia
Absolute eosinophilic count>350
Pts with hemolytic anemia have high ___ bilirubin
Unconjugated
In adult pt with direct hyperbili and high alk phos, what should you do first
Abdo us
Elevated c peptide levels and proinsulin levels are seen in pts with ___ tumors
Beta cell
Multiple myeloma is a infiltration of the ____ leading to ineffective ___ production
Bone marrow
Antibody
What is angiodysplasia? What are associated conditions?
Painless gi bleeding with dilated submucosal veins and av malformations
Associated with renal dz, vwd, aortic stenosis
B thalassemia is a ___opathy
Hemoglobinopathy
With pts who have a dvt/pe but can’t be anticoagulated what do you do?
Inferior vena cava filter
Can you get steatorrhea, ftt and vitamin deficiencies in cystic fibrosis
Yes
Can see bruising
What are the two kinds of acute leukemia’s
AML and all
Etiology of wiskott Aldrich syndrome
Impaired cytoskeleton changes in leukocytes, platelets
Eczema, microthrombocytopenia and recurrent infections in a toddler/baby, think
Wiskott Aldrich
Abscesses due to fungi or cat positive bacteria is the characteristic features of what immunology dz
Chronic granulomatous dz
Etiology of Chronic granulomatous dz
Inability of phagocytes to produce hydrogen peroxide in their lysosomes
__ of donor blood can prevent febrile nonhemolytic reaction and reduce risk of __ and ___
Leukoreduction
Hla alloimmunization and cmv transmission
Reticulocytes are __ in aplastic crisis and ___ in splenic sequestration
Decreased
Increased
In a pt with scd, an acute drop in hemoglobin and a low retic count without splenomegaly is consistent with
Aplastic crisis
Which causes pancytopenia, aplastic anemia or aplastic crisis
Anemia
Most common complication in a person with sickle cell trait
Renal issues specifically painless hematuria or hyposthenuria (inability to concentrate urine)
Are low molecular weight heparin and rivaroxaban recommended in pts with esrd
No because they are metabolized in the kidney
Is unfractionated heparin contraindicated in pts with esrd
No
Pica is a behavior that sometimes accompanied
Iron deficiency anemia
What long term meds should you take to prevent stent thrombosis
Aspirin and platelet blocker (clopidogrel)
Strongest indicator of stent thrombosis in first twelve months after placement
Premature discontinuation of antiplatelet therapy
Peripheral blood smear in autoimmune hemolytic anemia may show
Spherocytes without central pallor
Why do you get arterial and venous thrombosis with hit?
Hit antibodies activate platelets resulting in platelet aggregation
What causes warfarin-induced skin necrosis
Rapid decline in protein c levels
Scc of the mucosa of head and neck is common in pp with a sig hx of
Alcohol and tobacco use
Scc positive lymph node in a person with a sig hx of alcohol and tobacco use-what initial test should you do?
Triple endoscopy-esophagoscopy, bronchoscopy, laryngoscopy
Aka panendoscopy
If a cervical lymph node in a woman shows metastatic adenocarcinoma what cancer should you consider
Breast
Treatment of mild hypercalcemia in setting of metastatic cancer to the bone
Bisphosphonates
Radiation therapy can be used for severe pain
You are thinking multiple myeloma. What should you get?
Spep
Spherocytosis occurs due to defect in
Ankyrin gene
Coombs rest in hereditary spherocytosis is
Negative
LAb findings in hereditary spherocytosis
Increased mean corpuscular hemoglobin concentration, neg Coombs, increased osmotic fragility and abnormal eosin-5-maleimide binding test
In hereditary spherocytosis you have __ mean corpuscular volume and __ mean corpuscular hemoglobin concentration
Low
High
Tx of hit
Stop all heparin products, start a direct thrombin inhibitor (argatroban) or fondaparinux
Iron and ferritin levels in thalassemia
High
Tibc in thalassemia
Low
First finding in peripheral smear of iron def anemia
Anisocytosis (rbcs of unequal size) which causes increased red blood cell distribution width
Do you see hypochromia in peripheral smear in iron def anemia
Yes
Abnormal lymph node is bigger than
2cm
Etiology of factor v Leiden
Ad point mutation in gene for factor v that makes it unable to respond to activated protein c
Most common cause of liver mass
Metastatic diseAse
Hepatic angiosarcoma presents in what demographic
Old men who have been exposed to toxins
Hepatic adenomas present in what demographic?
Young women
Hepatic adenomas May cause ___ and are associated with ___ use
Ruq
Ocp
Hepatocellular carcinoma typically emerges from ___ liver
Chronically inflamed liver
You find rectal cancer. What to do next?
Colonoscopy to the cecum
In splenic sequestration crisis reticulocyte count in __ and platelet count is __
Up
Down
Peripheral blood smear in folate or cobalamin deficiency shows
Late rbcs with hypersegmented neutrophils
Methylmalonic acid in cobalamin def is __ and in folate def is ___
High
Normal
Deficiencies in cobalamin and folate will cause ___ homocysteine levels
High
What age range besides Childhood do craniopharyngiomas present?
Age 55-66
Most significant environmental rf for pancreatic cancer
Smoking
Most common kind of pancreatic cancer
Adenocarcinoma
Lab findings in pancreatic carcinoma
High serum bili and alk phos plus mild anemia
Multiple liver lesions on ct. where is the cancer?
Likely metastatic diseAse, most commonly from Colon. Do a colonoscopy
Red cell distribution width in:
Iron def
Alpha thalassemia
Beta thalassemia
Iron def-high
Thalassemias- normal
Red blood cell count in:
Iron def anemia
Alpha thalassemia
Beta thalassemia
Low in iron def
Normal in thalassemias
Peripheral smear in alpha and beta thalassemia
Target cells
Thalassemias response to iron supplementation
No improvement
Massive painless hematuria in scd withoht other sx is auspicious for
Papillary necrosis
Two common complications of hereditary spherocytosis
Pigment gallstones and aplastic crisis
Splenectomy can cause a dramatic __ in platelets
Rise
Two common complications of hereditary spherocytosis
Pigment gallstones and aplastic crisis
Splenectomy can cause a dramatic __ in platelets
Rise
People without a spleen have what kind of bodies in their rbcs
Howell jolly bodies
In seminomas, bhcg is __ and afp is ___
High
Normal
First line tx for chemotherapy induced nausea
Serotonin 5ht receptor antagonists (eg ondansetron)
Role of haptoglobin
Binds to free hemoglobin and promotes its excretion
Tibc in anemia of Chronic disease is high or low?
Low
High homocysteine levels can be normalized by admin of what two things
Pyridoxine b6 and folate
Three causes of malignancy in young men
Testicular cancer
Lymphoma
Leukemia
What kind of contraception should you use in pts with breast cancer
NOT hormone containing contraception
Use for example copper iud