Hematology Flashcards
What is a complication of chronic anemia?
High cardiac output failure
After diagnosing anemia either with a low hematocrit or low hemoglobin what is the next step in managment to determine the kind of anemia?
MCV
Normal MCV
80-100
What is the heme component of hemoglobin ?
portyprhrin and Fe
What is the globin component of hemoglobin?
Alpha and Beta proteins
What laboratory value should be checked in a patient with anemia (low hematocrit or hemoglobin), but a normal MCV?
Reticulocyte
Patient with anemia with a normal MCV either are anemic because increased _______ or reduced ______ of RBC’s
Destruction or production
What labs would we reference in order to differentiate, between intravascular and extravascular hemolysis?
Haptoglobin, LDH, Bilirubin, Hemoglobin in the Urine.
What would haptoglobin, LDH, bilirubin, hemoglobin in the urine look like in a patient with intravascular hemolysis in comparison with extravascular hemolysis.
Haptoglobin: low
LDH: high
Bilirubin: high
Hemglobinuria: present
splenomegaly: absent
What would haptoglobin, LDH, bilirubin, hemoglobin in the urine look like in a patient with extravascular hemolysis.
Haptglobin: normal to low
LDH: normal to high
Bilirubin: high
Hemoglobinuria: absent
splenomegaly: present
What are the causes of cold autoimmune hemolytic anemia
Mycoplasma pneumonia, EBV, WM, and lymphomas
What are the causes of warm hemolytic anemia?
SLE, drugs that cause SLE like symptoms, hemolytic anemia of a newborn.
A coomb’s test would be positive in autoimmune hemolytic anemias, what are the differences in the coomb’s test between warm and cold?
Cold: c3b and warm: Anti-G
What is the best screening test for spherocytosis?
eosin- 5 malemide, osmotic fragility test, acidified glycerol lysis, cryohemolysis
What does the blood smear show?
RBC’s w/o central pallor, which can be spherocytes, or autoimmune hemolytic anemia
What is the difference between autoimmune hemolytic anemia and spherocytosis?
Spherocytosis will be negative for Coomb’s. Although they correlate with most everything else. with IgG antibodies and being extravasular hemolysis
What are risk factors for G6PD
Drugs like sulfamethazole, primaquine, quinine, dapsone, isoniazid, and rifampin and fava beans
How do we diagnose G6PD
G6PD enzyme assay
What does this blood smear show?
Heinz bodies
What kind of anemias hemolyze intravascularly?
PNH, Cold agglutinins, G6PD, (also TTP, HUS, DIC)
What kind of anemias hemolyze extravascullarly?
Spherocytes, warm agglutinins,
What laboratory value should be checked in a patient with a MCV >100?
Folate or B12
A patient presents with to your office with complaints fatigue and of dark urine upon waking in the morning. What is the pathology behind this patients dark urine?
The patient has a genetic defect that makes his RBC membrane (PIG-A), which stands for decay acceelerating factor (CD55 and 59). In acidic environments are RBC’s become more susceptible (night time).
What is the most common cause of death in a patient with paroxysmal nocturnal hemoglobinuria?
Thrombosis
How does one diagnose paroxysmal nocturnal hemoglobinuria?
flow cytometry looking for CD55/59, also ham’s test and a sugar water test
What is the treatment for a patient with paroxysmal nocturnal hemoglobinuria?
Blood transfusion, in cases of persistent hemolysis consider prednisone, and if the presentation is severe consider ecluzimab
What is the amino acid change in sickle cell?
Glutamic acid –> valine
What are events that precipitate sickling>
ACTED –>RIGHT, Acidosis, high CO2, increased Temp, Elevated 23DPG,. And also of course low O2 environments like high altitude
Although a blood smear can be used to visulaize sickle cell what is the definitive diagnosis
hemoglobin assay would show >40% HbS if a patient has sickle cell if it’s less than 40 % then the patient has the sickle cell trait.
What are some of the complications of vaso-oclusive sickling crisis?
Acute chest syndrome, Splenomegaly, priapism, dactylitis, stroke, renal infartcion
What is the most common cause of osteomyelitis in a patient with SCD?
Staph Areus, although salmonella is more prevalent in patient with SCD Staph. A is more common.
True/False patient with SCD should get prophylatic antibiotics?
True & Falsee starting at the age of three months penicillin should be intiated for at least a year or until the age of 5. Adults do not require antibiotic prophylaxis
Vaccines should cover what type of organisms in a patient with SCD?
Encapsulated organisms
This drug increase HbF so that the patients RBC’s, hold on to oxygen and thus don’t sickle?
Hydroxyurea
All patients with chronic hemolysis should be started on what vitamin?
Folate
A patient f/u after labs identify that the patient has anemia. On examination of the labs the reticulocyte count is low what is the next step in management?
Get a bone biopsy, the fact that the reticulocyte count is low is suggestive that there is low bone production.
How do we differentiate normocytic anemias?
Normocytic is divided in to production and destruction. The next thing we need to ask ourselves is about the reticulocyte count? The destruction side will have elevated reticulocyte count. The production side will have a low reticulocyte count.
Name pathologies of the bone marrow that result in reduced production of hematologic precursors?
Red cell aplasia, Aplastic crisis, and myeloproliferative disorders ***
what are some of the causes of aplastic crisis?
Drugs: carbmazapine, anti-thyroid rx
Infections: Parvo, EBV, and HIV
When should we transfuse a patient?
when Hb is < 7-8, or 8 to 10 in a patient that is symptomatic
Iron deficeny?
- MCV
-Ferritin
-TIBC
-transferrin saturation
MCV: Low <80
Ferritin: Low
TIBC: High Transferrin: high
Transferrin saturation: low
Anemia of chronic disease
-MCV
-Ferritin
-TIBC
-transferrin saturation
MCV: Low <80
Ferritin: High
TIBC: low Transferrin: low
Transferrin saturation: low
Thalasemia
-MCV
-Ferritin
-TIBC
-transferrin saturation
MCV: low
Ferritin: normal
TIBC: normal
transferrin saturation: normal
RDW= normal
Sideroblastic anemia
-MCV
-Ferritin
-TIBC
-transferrin saturation
MCV: low
Ferritin: high or normal
TIBC: low Transferrin: low
What is the treatment for a patient with iron deficiency anemia?
Iron (ferrous sulfate)
What is the most common cause of iron deficency in a patient less that 50 years
menorrhagia
What is the most common cause of iron deficiency anemia in a patient > 50 year of age.
colon cancer
_____ is a feedback that shuts down transferrin, when Fe stores are high?
Ferritin, which is why TIBC and ferritin are inversely related.
What are the causes of sideroblastic anemia?
alcohol, B6 deficency, cooper or lead posioning
How many genes must a patient lose in a alpha thalassemia for the patient to become significantly symptomatic?
3 genes = alpha thalassemia
4 genes= Hb Bart’s, this child will probably not make the first few days of life
How many genes must a patient with beta thalassemia lose in order to be symptomatic
1= Beta thalassemia minor
2= Cooleys anemia (Thalassemia major)
Target cells are associated with what heme disorders?
hemoglobin C, sickle cell, and thalassemia
When does adult hemoglobin start replacing fetal hemoglobin?
At 6 months
What is HbA hemoglobin?
It contains two alpha globins and two beta globins. Its is the hemoglobin primarily found in adults
A patient with anemia has normal iron studies and normal RDW, what is the next step in managment ?
Suspect thalassemia and get a hemoglobin assay
What is HbA2 hemoglobin
It’s two alpha chains and two delta chains comprises of only 2% of adult hemoglobin.
What is HbF hemoglobin?
It’s two alpha and two gamma chains and this is most prevalent in infants
What is a complication of regular blood transfusions?
Hemochromatosis
_____ is an autoimmune disease that attack parietal cells. Patients may complain of loss of neuropathy, dorsal column nerve deficits.
Pernicious anemia
How could pancreatitis cause a megaloblastic anemia?
exocrine pancreatic deficency results in reduced binding of B12
What drugs can precipitate a megaloblastic anemia?
methotrexate. TMPSMX, 6 mercaptopurine, phenytoin
Where is INF and B12 reabsorbed?
in the illeum
What are the etiologies of B12 deficiency?
Food ingestion, Food digestion (stomach, ileum, pancreas), malabsorption
How does B12 deficiency cause toxicity to the nervous system?
Methylmalonic acid accumulation
What is the MCC of folate deficency?
Alcoholism
What does LAP stand for?
Stands for leukocyte alkaline phosphatase
Basophilic stipling can present in what other blood pathologies?
Thalasemia, alcohol use disorder, vitamin deficiencies and lead poisoning.
A 31 yo F patient is scheduled for an emergency surgery for appendectomy. Prior to the surgery her platelet count was normal, but her PT and PTT are elevated. A 1:1 inhibitor mixing study is done (pts plasma is mixed with normal plasma), but her PT and PTT haven’t corrected what could be the possible cause?
lupus anti-coagulant a form of anti-phospholipid antibody. It’s the most common form of coagulant inhibition.
A 5yo child presents with acute onset of fatigue. The patient has a previous history of sickle cell. The patients hemoglobin is 6. The patient is slightly dyspenic, but responsive to simple commands. The patients spleen is palpable. Reticulocyte count is elevated, what is the cause of her reduced hemoglobin levels?
The patient has splenic sequestration which is a complication of an occlusion of an exit route from the spleen thus causing RBC’s to become trapped in the spleen. The next step would be to give this patient a transfusion.
Shiny tongue and palmar creases may be incognito to suggest?
A vitamin B12 deficiency
What’s the treatment for a patient with TTP?
Plasma transfusion
Would this lab suggest ITP or Von Willebrand’s dz
Remember that while Von Willebrand will increase PTT and BT, it usually doesn’t affect the platelet levels. So this lab suggests more ITP.
A patient of good stature, erratic behavior present with these labs. Would this lab be more suggestive of EPO abuse or steroid abuse?
This lab would be more suggestive of steroid abuse, steroids can cause erythocytosis. In addition, if the stem suggests increased aggression and gynecomastia consider steroids.
A pt. with a hx of Sjogren’s autoimmune, shiny tongue, and IBS
Megaloblastic anemia due to pernicious anemia.
If a patient is in an emergent situation and you don’ t have the time to crossmatch blood, what blood should you give?
Blood group O, Rh (-)
A pt. after blood transfusion is has a b/p 80/50, HR 120, the patient has labored breathing on physical examination. What kind of transfusion reaction is she having?
Anaphylatic
A pt after blood transfusion is short of breath, CXR show pulmoary infiltrates and edema, JVD is absent, PCWP <18 what transfusion reaction is she having?
TRALI (transfusion related acute lung injury)
Patient that experiences fever and chills 20 mins after a blood transfusion, what is the cause of fever?
Febrile non-hemolytic; cytokine release during blood storage. Test my quiz you on doing ABO compatibility to make sure that this isn’t ABO incompatibility.
What is an allergic/urticarial reaction
Although it can present with similar symptoms to anaphylaxis there is no shock presentation. The patients IgE antibodies cross react and cause mass cell degranulation.
A blood transfusion that causes anaphylaxis is due to lack of exposure to what anti-body?
IgA
A patient hours after a blood transfusion has dark urine, is what type of transfusion reaction?
Acute hemolytic reaction
A patient after blood transfusion has dyspnea, CXR reveals opacities, JVD, elevated BNP.
TACO (transfusion associated cardiac overload)
A patient after blood trasfusion has dyspnea and CXR show opacities, with a PCWP< 18?
TRALI
How can we prevent a febrile hemolytic reaction from occurring?
Leukoreduced blood
What kind of reaction is graft versus host disease
This is a cytotoxic (CD8) reaction that typically develops by day 100. Donor CD8 cells respond to recipient antigens. Typical patient presentation is GI symptoms like diarrhea or maculopapular rash. The diagnosis is made by biopsy and treatment is usually steroids.
What’s the management for splenic sequestration.
The first goal is packed RBC transfusion. If the patient were to have greater than one incidence of splenic sequestration than we might consider splenectomy.
What are some signs of splenic sequestration?
Hypotension, splenomegaly, hepatomegaly, low hemoglobin, thrombocytopenia, reticulocytosis
What’s the difference between WM and MM?
A patient that is 40 yo with chronic bilateral MCP joint pain and stiffness presents to your office in search of possible treatment. Patient has had X-rays in the past showing calcifications within the joint space. Patient had recent lab work that showed AST 100 and ALT 105? What is a possible diagnosis?
Hereditary hemochromatosis
What’s the treatment for hereditary hemochromatosis?
NSAIDS, but also sometimes phlebotomy
What are myeloproliferative disorders?
They are overproductions of the bone marrow
This disease presentation is the proliferation of all myeloid lineage mainly granulocytes?
CML
_____ is the proliferation of RBC’s
Polycythemia vera
All myeloproliferative disorders have a _____ mutation?
JAK-2
What is the definitive diagnosis for myeloproliferative disorders
Bone marrow biopsy
C-abl, t9:22, tyrosine kinase activity increases generation of myeloid cells
CML
What is the treatment for a patient with CML
Tyrosine kinase inhibitors; imatinib, - -inib
What is the major complication of CML?
it can progress to a blastic crisis and transform into AML/PML
What are common lab findings for acute leukemia?
elevated blasts (>20%), low platelets, low WBC’s, low RBCs
How do we categorize leukemias?
Leukemias are first divided into acute and chronic. Then leukemias are classified as myeloid or a lymphoid
What is the demographic of patients AML
middle age
What is the demographic for patients with ALL
Children
What are three unique features of PML?
DIC, Auer rods,
What is the treatment for PML
Vitamin A
What is the genetic translocation for PML?
15;17 with an abnormal retinoic acid receptor
What is the first step in diagnosis if you suspect a patient has leukemia?
Blood smear will give us an indication if the leukemia is acute or chronic bast on the presence or absence of blast cells
What are some of the complications of tumor lysis syndrome
hypocalcemia (remember that Ca binds PO3) hyperuricemia causing gouty arthritis.
How does chronic lymphoid leukemia present like?
chronic leukocytosis
How do we diagnose CLL?
flow cytometry
Flow cytometry + CD11/22 , TRAP, and dry TAP
Hairy cell leukemia
protein electrophoresis with polyclonal spikes indicate?
chronic inflammation
Protein electrophoresis with monoclonal spike indicated what kind of pathologies?
WM, MM, Monoclonal gammopathy of undetermined significance (MGUS)
What is the difference between MGUS and MM?
in MGUS the M3 <3% and the plasma cells are <10%
All plasma cell disorders make monoclonal proteins causing this RBC formation on the slide.
Rouleaux
What are the complications of plasma cell disorders?
Hypercalcemia (IL-1 activates osteoclasts) nephrotoxicity (light chain deposition), anemia (with many blood disorders overcrowding of the bone marrow leads to decreased production of normal cells), infection
What is the MCC of death in MM?
Infection
Why is it important for patients with plasma cell disorders to get vaccinated?
Remember they are not producing normal B cells and the dysplastic B cells are overcrowding normal B cell s
Lymphomas are organized into?
Hodgkins and Non-Hodgkins
What are B symptoms?
fever, drenching night sweats, and weight loss
Patient with fatigue and 12lb weight loss in 6 months has flow cytometry + CD15/33 and binucleated cells.
Hodgkin lymphomas
What are risk factors for Non-Hodgkin Lymphomas
EBV
How do we diganose someone we suspect has a lymphoma?
Lymph node biopsy
What findings are concerning for a malignant lymph node?
fixed, >2cm, and immobile
Increased bleeding time, normal platelet production, ristocetin =0, very large platelets?
Bernard soluier
Increased bleeding time normal platelet production, ristocetin varaible and PTT increased?
VWF
When is platelet transfusion indicated in a patient with ITP (immune thrombocytopenia).
platelets are <10,000
Renal failure in a child, hematuria, and low platelets?
HUS
Low platelets, low adams t13
TTP
Patient presents with low platelets elevate PT and PTT and has oozing of blood from a catheter site.
DIC
A patient with elevated PTT undergoes a mixing study which normalizes his/her coagulation studies what is the next step in determining what factor was deficent?
specific factor assay
A patient comes to the office with abnormal coagulation studies. The patient has a history of pancreatitis, what vitamin dedicency
Vitamin K
vitamin K is a cofactor for what clotting factors?
2,7,9,10
What is the MOA of heparin
It activates anti-thrombin 3 which is an inhibitor of Xa
After four days on heparin therapy a patient presents with thrombocytopenia and elevated PTT what is the MOA?
IgG binds to heparin/platelet complex 4
What is the treatment for a patient with HIT 2?
Stop drug and anticoagulation with agotroban
Patients with gout have hyperuricemia which can cause
thrombocytosis
What is the treatment for enterobiasis (pin worms of the ass)
In pregnant women Pyrantel pamoate, in non- pregnant patients -bendazoles
What is the most common inherited thrombophilia?
Factor 5 leiden, Factor 5 essentially inhibits Protein C which is an anti-coagulant. Look for a young male patient with venous thromboembolism with no risk factors .
