MTB - Hematology Flashcards
In older patient with anemia - what level of Hb is your goal?
Above 10
Iron studies in anemia of chronic disease
High ferritin
Low TIBC
Low Fe
Normal/low Fe sat
Electrophoresis in B thalassemia
Elevated HbA2 and HbF
Hb electrophoresis in A-thalassemia
Normal
What are potential causes of sideroblastic anemia?
Alcoholic
Lead exposure
Isoniazid
Most accurate diagnostic test for sideroblastic anemia
Prussian blue stain
Treatment of sideroblastic anemia
- Remove toxin exposure
2. Pyridoxine replacement
Most accurate test for alpha thalassemia
DNA sequencing
Which anemia has increased RDW?
Iron deficiency only
Only microcytic anemia with high reticulocyte count
HbH - 3 alpha gene deletion
Anemia of chronic disease
Normal or increased amounts of iron and ferritin in storage but inability to process the iron into a usable form for Hb synthesis
EPO can be used to tx what anemia?
Anemia of chronic disease due to end stage renal disease
Three unique signs of vit B12 deficiency
Neuropathy (peripheral)
Glossitis (smooth tongue)
Diarrhea
Which drug can block vit B12 absorption?
Meteor in
Peripheral blood smear findings in megaloblastic anemia
Hypersegmented neutrophils (>4 lobes) Oval cells
What other lab tests should you get in macrocytic anemia?
Bilirubin and LDH - will be increased
Reticulocyte count - will be decreased
Your suspect vit B12 deficiency in your patient with macrocytic anemia, but their b12 level is normal - what other test can help you distinguish between vitamin B12 and folate deficiency?
Methylmalonic acid level - will be elevated in B12 def
Homocysteine is elevated in both
You found low vitamin B12 levels and elevated methylmalonic acid levels in your patient - what’s the next test to determine etiology of deficiency?
Anti-parietal cell abs or anti- intrinsic factor abs
Lab findings in hemolytic anemia
Elevated bilirubin, LDH and reticulocytes
Decreased haptoglobin
In addition to regular findings of hemolysis, intravascular hemolysis shows…
Schistocytes, helmet cells and fragmented cells on smear
Hemoglobinuria
Hemosiderin urea
A patient with sickle cell anemia presents with severe pain in the chest, back and thighs. What is your initial management?
Oxygen
IVF with normal saline
Pain meds
Patient with sickle cell crises, develops fever while on oxygen and hydration. What do you do?
Give abx: ceftriaxone, levofloxacin or moxifloxacin
Perform complete physical exam
Order blood cultures, urinalysis, reticulocyte count, CBC and CXR
In what cases would you give an exchange transfusion to a sickle cell pt
Visual disturbance
Pulmonary infarction
Priapism
Stroke
Sickle cell pt admitted for pain crisis has a drop in htc with rise in reticulocyte count over two days - what do you suspect?
Either folate deficiency or parvovirus infection
If pt is receiving replacement therapy, it is likely parvo
Dx and Tx of parvovirus in sickle cell pt
PCR for DNA of parvovirus
Tx, transfusion, IVIG
What meds do you discharge a sickle patient?
Folate replacement
Pneumococcal vaccine
Hydroxyurea - if >4 episodes per year
CF in sickle cell trait
Renal problems
- hematuria
- isostenuria (prone to dehydration)
- urinary tract infections
Causes of autoimmune hemolysis
Other autoimmune conditions:SLE, RA
CLL
MEDS: penicillin, alpha-methyl dopa, quinine, sulfa drugs
Most accurate diagnostic test in autoimmune hemolysis
Coombs test
Best initial therapy in autoimmune hemolysis
Steroids (prednisone)
If recurrent - splenectomy
Only IgG abs respond to these treatments
If a case describes severe hemolysis that does not respond to prednisone or repeated transfusions - what can you do?
Give IVIG
Features of cold induced hemolysis
Mycoplasma or EBV in history
Coombs negative
Complement positive
No response to steroids, transfusion or IVIG
Tx. Cold hemolysis
Rituximab
Which anemia is X-linked?
G6PD Deficiency
Causes of acute oxidant stress in g6pd deficiency
Infection
Sulfa drug, primaquine, dapsone
Fava beans
Best initial test in g6pd deficiency
Heinz body test - Heinz bodies and bite cells on smear
Most accurate test for G6PD deficiency
G6pd level - wait two weeks after acute episode to measure level (will be normal right after episode )
Tx. G6PD deficiency
No tx - avoid oxidant stress
CF in hereditary spherocytosis
Recurrent episodes of hemolysis
Splenomegaly
Bilirubin gallstones
Elevated MCHC
Causes of HUS / TTP
E.coli O157:H7
Ticlopidine
HUS triad
Hemolysis
Renal failure
Thrombocytopenia
TTP
Hemolysis Renal failure Thrombocytopenia Fever Neurological changes
Tx. HUS / TTP
Plasmapheresis
Patient is presenting with pancytopenia and recurrent episodes if dark urine - dx? Test?
Dx: PNH
Test - c55 and c59 ab level
Tx. PNH
Steroids
If transfusion dep. try eculizumab
What drugs can cause methemoglobinemia?
Nitroglycerin Amyl nitrate Nitroprusside Dapsone Anesthetics ending in a sine
Tx. Methemoglobinemia
Methylene blue
Acute symptoms of hemolysis while transfusion is occuring
ABO incompatibility
Acute SOB shortly after transfusion that goes away on its own
Transfusion assoc. Acute lung injury
- donor abs against WBCs
- no tx
What transfusion reaction presents with delayed jaundice and no other symptoms?
Minor blood group incompatibility
No specific therapy
Best initial test if you suspect leukemia
Peripheral smear - will show blasts
Most impt prognostic test in leukemia
Cytogenetics ex. Karyotypic analysis
Tx. AML
Chemotherapy with idarubicin and cytosine arabinoside
What extra tx. Is given in M3 AML
All trans retinoic acid
Additional therapy for ALL
Intrathecal methotrexate
Leukostasis as the initial presentation of acute leukemia
Slushing of blood in blood vessels of eyes, brain and lungs when WBC is > 100,000.
Tx. Leukostasis
Leukaphoresis
Hydroxyurea
Pelger-Huet cell
Neutrophil with two lobes seen in myelodysplasia
Pt presents with pancytopenia, elevated MCV, low retic count and macroovulocytes with Pelger Huet cells. Dx?
Myelodysplasia
Tx. Myelodysplasia
Supportive, transfusions as needed
Azacitadine - specific therapy
Lenalidomide
Drug approved for 5q minus syndrome form of myelodysplasia
Pt presents with elevated WBC predominantly neutrophils and splenomegaly - dx?
CML
Best initial test in CML
LAP score
- elevated neutrophils but LOW LAP
Most accurate test for cml
Philadelphia xm
Tx. CML
Imatinib (also can use: dasatinib or nilotinib)
BMT only if no response to medical therapy
55 year old patient presents with elevated WBC on routine CBC. It is predominantly lymphocytes. Dx? Best initial test?
Dx. CLL
TEST: peripheral smear will show smudge cells
Tx. CLL
Based on stage
Stage 0-1: elevated cells, enlarged LN - no therapy
Stage 2-4: splenomegaly, anemia, low platelets - fludarabine + rituximab
Alemtuzumab
Anti CD52 AB used in tx of CLL
55 yo pt presents with pancytopenia and massive splenomegaly. dx? Test?
Dx. Hairy cell leukemia
Test: trap stain, peripheral smear shows hairy cells
Tx. Hairy cell leukemia
Cladribine (2-CDA)
55 yo patient presents with pancytopenia and splenomegaly. TRAP stain is negative. Smear shows tear drop cells. Dx? Tx?
Myelofibrosis
Tx. BMT
If BMT is not possible, best initial therapy is Lenalidomide or thalidomide
Lab findings in polycythemia Vera
Elevated Htc, WBC and platelets
Decreased MCV and EPO
A patient has high hematocrit on CBC, what test do you order next?
Arterial blood gas
- want to rule out hypoxia as a cause of erythrocytes is
EPO level
Jak2 mutation
Tx. Polycythemia Vera
Phlebotomy
Hydroxyurea - can decrease red count
Daily aspirin
Tx. Multiple myeloma
Melphalan and steroids
Most effective therapy for multiple myeloma
Autologous BMT
Prognostic indicator in multiple myeloma
Beta 2 micro globulin level
MGUS
Asymptomatic elevation of IgG in elderly person with total elevated protein. No treatment
Pt presents with blurry vision, confusion, headache, enlarged LN and spleen. IgM levels are elevated - dx
Waldenstrom macroblobulinemia
Best initial test in Waldenstrom macroglobulinemia
Serum viscosity test or SPEP
Best initial therapy for Waldenstrom macroglobulinemia
Plasmapheresis
Best initial test for Hodgkins and NHL
Excisional lymph node biopsy
Chemo tx for hodgkins
Adriamycin
Bleomycin
Vinblastine
Dacarbazine
Chemotherapy for NHL
Chop regimen Cyclophosphamide Hydroxyadriamycin Oncovin (vincristine) Prednisone
Pt presents with superficial bleeding from skin and mucosal surfaces. Platelet count is normal. Bleeding gets worse every time they take aspirin. Dx?
Von willebrand dz
Most accurate test for Von willebrand dz
Ristocetin cofactor assay and vWF level
First line tx of Von willebrand dz
Desmopressin or DDAvp
- releases subendothelial stores of vWF and factor VIII
Sudden appearance of rash and or bleeding in otherwise healthy child
Consider ITP
What antibodies are present in ITP
Glycoproteins IIB/IIIa receptor abs
Tx. Mild ITP (mild bleeding with platelets <50,000)
Prednisone
Tx. ITP with platelet count <20,000 and serious bleeding (intracranial hemorrhage)
IVIG or Rho gam
What drugs can be used in treatment of ITP if no response to splenectomy and still having recurrent episodes?
Romiplostim
Eltrombopag
Tx. Uremia induced platelet dysfunction
Desmopressin
Prolonged aPTT and bleeding that does not correct with a mixing study
Factor VIII antibody - MCC
Pt who was given heparin three days ago for DVT prophylaxis comes in a new thrombosis - what should u be considering?
Heparin induced thrombocytopenia - drop in platelets few days after heparin treatment; thrombosis is most common clinical manifestation
Best initial diagnostic test for HIT
Platelet factor 4 abs
Heparin induced anti platelet abs
CCS - what physical exam stuff do you order for suspected anemia?
General appearance, cv, chest, extremities, HEENT
Best initial therapy for HIT
Stop heparin
Give direct thrombin inhibitor (argatroban, lepirudin)
Tx. HIV associated thrombocytopenia
Zidovudine
- min 600 mg/day
Corticosteroids will work but will recur after stopping them and can increase risk of opportunistic infections
If an HIV positive patient adheres to a 3 drug HARRT regimen - when will their viral load decrease to < 50 copies/ml?
16-24 weeks
- measure viral load at 4 weeks, 8-12 weeks and then remeasure every 6-8 weeks thereafter
tx. of inoperable head and neck cancer
combination chemo-radiotherapy
Type 1 HIT
w/in 2d of initiation of heparin therapy; platelet count returns to normal with d/c of therapy
Type 2 HIT
abs against heparin platelet factor 4 which causes platelet activation and aggregation leading to thrombocytopenia and platelet rich clots; usually 4-10 days after initiation of heparin therapy and can lead to arterial or venous thrombosis
warfarin induced skin necrosis
high dose warfarin induces a transient hypercoagulable state by causing rapid reduction in protein C levels on the first day of therapy; lesions on breasts, trunk and extremities. No thrombocytopenia
heparin induced skin necrosis
complication of unfractionated heparin; involves areas rich in fat
Tx. in HIT
discontinuation of all heparin products and warfarin and start anticoagulation with direct thrombin inhibitors (lepirudin, argatroban)
limitations of lepirudin and argatroban
lepirudin - used in caution in renal insuff pts
argatroban - use in caution with hepatic dysfunction
MCC of anemia in pts with ESRD
EPO deficiency
absolute iron deficiency
< 100 ng/ml
iron supplementation in pts with ESRD is recommended when
when transferrin sat < 30% and ferritin < 500ng/ml
- iron supp. should be IV for pts receiving dialysis for ESRD
pt with low Hb despite being on EPO, ferritin > 500 - next step/
increase dose of EPO
tx. of anemia of chronic disease in patients with RA
anti-TNF agents, i.e. infliximab
next step in eval of normocytic normochromic anemia
reticulocyte count
- to r/o hemolytic causes
major problem leading to difficulty finding cross-matched blood in patients with a history of multiple transfusions
alloantibodies - most commonly, E, L and K
indications for irradiated RBCs
BMT recipients
immunodeficiency pts
blood components donated by first or second degree relatives
indications for leukoreduced RBCs
chronically transfused pts
CMV seronegative at risk pts (HIV, transplants)
potential transplant recipients
previous febrile non hemolytic transfusion reaction
indications for washed RBCs
IgA deficiency
complement dep. autoimmune hemolytic anemia
continued allergic reactions with transfusion despite antihistamine treatment
initial diagnostic test for pernicious anemia
anti-IF ab testing
findings on biopsy in pernicious anemia
absent rugae in fundus and body–> autoimmune metaplastic atrophic gastritis
EPO levels in polycythemia vera
normal or low
polycythemia + high EPO levels
secondary causes
- consider getting nocturnal oximetry and carboxyhemoglobin
tx. multiple brain mets
whole brain irradiation
- can improve survival 3-6 months
screening while on tamoxifen
no additional screening needed
- annual gyn exam with complete history and routine pap smears
1 unit of platelets should increase platelet count by how much
5000 platelets
- measure post transfusion count 10-60 minutes following transfusion
platelet refractoriness
absolute platelet count increment of less than or equal to 2000 / unit of platelets given - MCC - alloimmunizatin (ab to HLA class 1 antigen on platelets)
