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155 Cards in this Set
- Front
- Back
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Normal WBC count
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4800-10,800/cmm
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Normal RBC count
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4.7-6 M/cmm
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Hgb normal value
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14-18
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MCV normal
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80-94
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Normal Platelet count
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143-365 k/cmm
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RDW normal
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how big of difference there is in cell size
11-15% normal |
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Elevated MCV with normal RDW
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B12 macrocytic anemia
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Initially decreased MCV and increased RDW?
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Fe-Def. Anemia
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Classic Fe Deff. Labs
-Hbg and Hct -MCV and mch -Often platelet count? -_____correlation with chronic? |
-Low
-Low Platelets high -GI or GU blood loss |
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Macrocytic Anemia Pattern to recognize?
-Hgb, and Hct -McV -Platelets -Clinical corr with diseases of? TQ** |
Low Hb and Hc
High MCV and normal or high mch. (Thrombocytopenia) -leading to nutritional deficiencies such as malnutrition, alcoholism, parasitic infestations**(tape worm***), and alterations in the intestinal tract. |
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Acute Blood Loss pattern
-Hgb and hct -mcv and mch |
-Low to normal
-Normal |
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Anemia of Chronic Disease/ Inflammation
-Hgb and hct -Mcv and MCH chronic examples |
low
Normal -COPD, Renal, Autoimmune, Cancer |
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one fo the most common causes anemia?
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#1 is Fe deficiency
chronic renal disease and diet |
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Pallor in anemia best seen where?
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palmar crease Probalby below 7 Hb
and pale conjuctiva |
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Hyperseg. nuetrophil think?
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B12 deficiency
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Synthetic liver test actually includes?
- - - |
Serum albumin
Platelet count PT-INR |
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bone marrow does not produce enough blood?
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aplastic anemia
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Primary Bone Marrow dysfunction two main cuases?
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Aplastic Anemia (drugs, tox, Infectiosn)
Myelophistic Anemia (cancers, tumors that crowd out normal cells.) |
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Normocytic Anemias list of main causes due to increased RBC loss or destruction?
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Acute Blood loss
Hypersplenism Hemolytic Disorders Aquired conditions |
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Normocytic Anemias due to decreased Production
Primary causes |
Bone marrow aplasia
Myelopathies Myeloproliferative disorders Pure red blood cell aplasia (Blackfan-Diamond syndrome) |
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Sideroblastic Anemia
What the flip is it? |
Lots of iron but you just cant use it!!
-Anemias due to poor bone marrow utiliztion of iron stores |
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Normocytic Anemia algorythim. Step 1... Reticulocytes increased?
Yes NO |
Yes----> Hemolysis or BLood LOSS
No---> Are there any other cytopenias or abnormal blood cells? Yes---> Bone marrow eval! NO? get better history for 2ndary cause |
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MCV=
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Mean Corpuscular volume
1) Microcytic (<80 μm3) ■(2) Normocytic (80-100 μm3) ■(3) Macrocytic (>100 μm3) |
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MCHC
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2.Mean corpuscular hemoglobin concentration (MCHC)
a.Average Hb concentration in RBCs |
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MCH
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mean corpuscular Hb
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Decreaed MCHC
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■(1) Correlates with decreased synthesis of Hb
■Example-all the microcytic anemias ■(2) Central area of pallor is greater than normal. ■Called hypochromasia (see Fig. 11-11) |
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First Aid list of Microcitic anemias
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MCV <80
Fe deficiency Thallaseimias (beta and alpha) Lead poison Sideroblastic anemia |
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Myelophistic Anemia
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Anemia due to a crowding out of the bone marrow
(cancers, tumors, metastasis and granulomas TB or sarcoid.) |
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First Aid list of Normocytic NON-hemolytic Anemias
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Anemia of chronic disease
APlastic Anemia Kidney Disease (Myelophsthitic included in our lectures) |
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FA list of NORMOcytic INTRINSIC HemolLYTIC anemias
(E= extravascular, (macrophage clears at spleen) (I=intravascular) |
Hereditary Spherocytosis (E)
G6PD def. (I) Pyruvate Kinase Def. (E) Sickle Cell (E) HbC disease PNH (paroxysmal nocturnal hemaglobinuria) (I) |
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1st step workup of microcytic anemia?
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Look at ferritin
low= Fe defficient High---> get hx for crhonic diease and/or CRP for inflammation no inflammation?--->consider thallasemia |
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Pt comes in with RDW with 75 and a normal/slightly low TIBC, low percent sat, and High ferritin. What you thinking?
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Anemia of chronic disease or inflamation
**High ferrritin** (total body iron storage) Fe deficiency anemia has a low ferrtin |
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Ferritin levels are usually____ in normocytic anemias and _____ in hemolytic anemias
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normal--high normo
HIGH in Hemolytic |
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Macrocytic Anemia
causes? - - - (First Aid List) |
-Megaloblastic Anemia
- Folate or B12 def. -Non Megaloblastic Macrocytic anemias - EtOH, Liver diseas, etc |
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Pt comes in with a loss of vibratiory sense and proprioception on your physical exam. They also have peripheral neuropathy, spasticity, and a mild dementia. There inintial complain was fatigue and short of breath. Lastly patient has mild glossitis. What are you thinking doc?
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Macrocytic Megaloblastic B12 deficiency anemia
**Posterior column signs (proprio and vibration) and other neurologic events due to lack of B12 in the fatty acid pathways needed for our fat CNS/ |
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Physical Sx in macrocytic anemias?
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GLossitis
Hyperpigmented Oral mucosa and Palmar crease (addisons) Hypopigmented hair Posteior Column signs spacticity dementia |
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When to suspect me some hemolytic anemia?
Labs? Smear? |
Hemolytic anemai following an oxidative stressor (Sulfa rx, Infxn, Fava)
Increased Retic count*** Increased hemolyisis labs (increased bili, epo, LDH) Heinzy bodies with bite cells Decreased haptoglobin |
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G6PD normal function
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Activates glutatione which is needed for
Methemaglobin----> Hemoglobin (*prevents Oxidative stress***) |
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GSPD deficiency cause?
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X-linked
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Heinz body is made from?
Two diseases to know? |
Hb denatures and binds to spectrin
G6PD deficency alpha thalasemia |
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G6PD Defieincy clinical pres?
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Back pain and hemoglobinuria a few days followin oxidative stressor.
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Hereditary Spherocytosis
Eitiology? |
Autosomal Dominant
Spectrin deficiency (less membrane so makes smaller more packed cells) Spherocytes |
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Pts comes into you for jaundice, splenomegaly and early cholelithiasis. On these clinical signs you suspect a hemolytic disorder. This disorder has an elevated MCHC. What are you thinking?
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Hereditary spherocytosis
get the smear for spherocytes |
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Tx for hereditary spherocytosis?
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Splenectomy
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Enhanced complement mediated hemolyisis from failure to inactivate compliment on RBC surface?
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PNH
(Black pee in the morning!) Increased urine hemosiderin |
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Autoimmune Hemolysis can be confirmed with what test?
What are treatments? - - - |
Coombs Test +
Tx -Prednisone -Splenectomy (not IgM) -Rituximab |
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What are the hemoglobinopathies?
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Sickle Cell Anemia
Thallesmia Hereditary persistence of fetal hemoglobin Aquired disorders |
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MCC of hemoglobinopathies
(example, Sickle cell) |
Point mutions
HbS (gAg to gTg) Glutamine to valine |
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Sickle Cell is an alteration in the ___chain of Hb?
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Beta
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Classic HbS disease findings? (labs that is)
- - - - |
Reticulocytosis
LDH elevation Haptoglobin Decline Bilruben Elevation |
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20 yo black male comes into your office presenting with severe tibial pain, without a hx of trauma. He recently had a streptococcal infection. What do you think the cause of his pain is?
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Most likely Vaso-Occlusive Crisis in sickle cell disease
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Crew cutt on a skull x-ray
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Thallasemias
and Sickle cell |
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Common agent responsible for osteomyltis in sickle cell?
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Salmonella
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Long Term Manegment in sickle cell?
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Hydroxyurea***
(first aid also includes BM transplant) |
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Thalassemias are the result of ?
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inadequate numbers of Normal tetramers of Hb.
(Quantitiative) |
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In beta thalasemia the alpha globin is____?
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in excess and TOXIC, and kills erythroblasts
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Facial(chipmonk)/ bone abnormalities, and a huge spleen. What are you thinking?
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Beta thalassemia major
(an other sign is Crew cut on x-ray) |
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Beta thal major look like on a smear?
- - - - |
**Target cells
Hypochromic Microcytic Nucleated RBC comon (same in beta minor however, just less) |
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HbH disease?
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Lost three alpha genes
Results in four beta globin tetramers being produced (HbH) |
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Hb Barts diease?
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will never see it bc die in utero! (NO APLHA genes)
-(Hydrops fetalis) Hg Barts is really four gammas (gg/gg) instead of alpha. This has a high affinity for oxygen and deprives the fetus. |
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Beta thal major treatment?
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Transfusion
Splenectomy (give pnuemovax) Chronic iron overload? Folate supliment Endocrine support |
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Thalessemia intermedia is what?
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one alpha (HbH disease) or beta 4 disease
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Suspect thalasemia however you must R/O what?
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Fe deficient anemia!
Thals have normal iron stores |
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Clott and bleed, clott and bleed?
(or Bleed bruise clinically) |
DIC
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Pathogeneis of DIC?
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Tissue factor gets into the blood which activates factor VII
*unopposed thromin----> clott *unopposed plasmin---> bleeder this is extrinsic pathway activation (must be a large enough amount to overwhelm the system) |
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DIC one of the most sensitive assays is for ?
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fibirn degredtaion products
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Sources of the tissue factor relaesed in DIC?
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Sepsis (Gram - bugs)
Solid tumors (pancreatic ex) Acute progranulocytic lukemia Burns Truama Snake bite Tissue Rejection Pregnancy problems Viral Other rare causes |
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Pregnancy complications that can cause DIC?
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Dead fetus
AMniotic fluid embolus Eclampsia HELLP syndrome |
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Clotting sx in DIC?
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DVT
PE Stroke Cardiac ischemia Renal Fail Hepatic fail |
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Bleeding Sx in DIC?
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Systemic signs
CUtaneous Urinary GI CNS |
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Increase in megakaryocytes and a defect causing the production of anti-GPIIb/IIIa antibodies .
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Idiopathic Trhombocytopenic Purpura
(TTP think vWF) |
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Deficiency of ADAMTS 13
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TTP
this is vWF metalloprotease deficiency witch leads to decreased degredation of vWF MULTIMERS THis causes platelet agregation and thrombosis |
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Labs for TTP?
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Schistocytes and increased LDH
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First Aid pneumonic for DIC causes?
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STOP Making New Thrombi
Sepsi (g -) Trauma Obstetric comp. Pancreatitis (acute) M= maliganncy N=Nephrotic Syndrome T=Tranfusioin |
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Labs for DIC?
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D-dimer** (fibrin degredation products) the best to confirm. (specific)
HOWEVER, he has undelined that tehre is Single test for it!*** Also see low plateslets, low fibrinogen etc. Clinical picture Schistocytes on smear |
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DIC treatmen?
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Identify cause and tx it!
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What is microangiopathic hemolytic anemia?
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Aquired anemia of many causes.
Hyperproliferative and has red blood cell fragmentation causing hemolysis. |
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Micropangiopathic anemai RBC profile?
(often) |
*Schistocytes (hemet cells)
Fragments Microspherocytes Burr cells **Increaed RDW** |
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Microangiopathic Hemolytic anemia causes?
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(seen in DIC, TTP-HUS, SLE, and malignanty HTN) or mechanical stressors, or infections ) first aid
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TTP sx classic five sx?
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Purpura
Microangipathic hemolytic anemia Neurolgic (strokes) Renal Abnormal (failure or bleed) Fever Due to clotting! |
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Disease is caused by Deficiency of ADAMTS 13?
explain the rest of the pathophys |
TTP**
this causes a decreased degredation of the vWF MULTIMERS. MULTIMERS cause increased platelet aggregation and THRMOBISIS and causes a low platelet count |
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DX of TTP?
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Purely CLinical Presentation
look for pentad (ADAMS TS13 available, order it to confirm however dont wait to tx!) |
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TTP tx?
Avoid? |
Plasmaphoreresis with plasma replacement
*** AND DO IT FAST if you suspect or high morbidity! (Removes the antibody to ADAMTS 13) NO PLATELET TRANFUSION** |
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3 year old comes into your clinic with a 3 day hx of bloddy diarhea that is clearing. The mother tells you she is worried because his urine is black! You notice the pallor of the child. What do you suspect?
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Childhood Hemolytic Uremia Syndrome (HUS)
2-5yo most common also comon to have CNS problems like drowsy or sezures |
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HUS
Pathophys? |
Ecoli or shigella produce shiga toxin, which causes the vascular damage.
This exoses the blood to the underlying tissue hence leading to coagulation activation |
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HUS vs. DIC how to lab ro DIC?
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Get a D-Dimer to RO DIC**
Normal clotting in HUS so D-DImer will not be elevated!! ALso HUS tends to be in children |
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Whats the adult tx for HUS?
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Start plasmaphoresis (bc you cant be sure its not TTP) untile your assays (ADMITS 13) get back!
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HELLP syndrome symptoms?
When common? Tx? |
Mailaise
RUQ pain HTN Proteinuria usually around 24-39 weeks Deliver parasite if can HTN control and eclampsia control |
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Signs of Thrombocytopenia?
- - - |
Purpura -cutaneous bleeding
Mucosal Bleeding- especialy epistaxis and gum Petechiae-pinpoint skin bleed |
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Normal Platelet count?
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150-450k
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1st workup in in platelet problem?
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CBC slip
(proably 1st in all blood problems) |
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Destruction of platelts
Autoimmune Causes |
ITP
SLE Pregnancy Drug related |
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Common thrombocytopenia causing RX?
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Heparin
Quinine |
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General Cause of ITP?
Specific causes |
anti GPIIb/IIIa antibodies therefore platelet destruction
(Idoptathic collagen vascular diseases Lymphomas Solid Cancers Drugs Viral (including HIV) |
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He says how to dx ITP is ?
- - - |
H &P
CBC Peripheral smear |
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ITP tx?
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If below 20k or bleeding like crazy
Dexamethasone Rituximab |
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HIT
Heparin Induced Thrombocytopenia what is it? |
Hypercoagulable State Arising from induction of antibody against heparin platelet factor 4 site
Antibody binds via Fab to Fc of another platelets cuasing it to release more PF4 (postive feedback!!) 4-10 days after exposed |
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HIT sx?
- * |
Thrombocytopenia (moderate)
*Arterial and Venous thrombosis |
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DX HIT?
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50% reduction in baseline platelet count EVEN if still in normal range
There is an assay to run and its good but again takes long so tx until results back |
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HIT tx?
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Stop Heparin
Anticoagulate (lepirudin or danapariod) DO NOT -start Coumadin (warfarin) or Platlet Transfuse! (duh they are alread cotting) |
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Multiparous female comes into your clinic 12 days after a blood transfusion from a ruptured placenta. She is bleeding, has a fever, and has purpura. What do you suspect and what is the pathophysiology of this problem?
What is the Dx and Tx? |
Post Transfussion Purpura
(Alloimmune type of thrombocytopenia) Usually P1 negative woman developes P1 antibodies to the blood that was given to her that has the antigen on it. Dx: can assay but she will be dead by the time you get it back so tx now on clinical. (<10,000 platelets usual) Tx: Supportive with plateltes from P1 Negative donor otherwise ** AVOID Transfusions |
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Platelet disorders that are Quality problems?
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Von Willebrand Disease
Glanzmans (no IIa/IIIb receptor) Bernard Soulier (no Ib-IX-V receptor) |
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Give Blood Hb must be?
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>12.5
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Whole Blood donation is about___mL?
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450-500
(250 packed RBC) (250 plasma) (30 platelets) |
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Infection screens in blood transfusions?
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HIV
Hep B Hep C Syphillis |
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RBC storage
Chemical? How long? (how long do platelets keep? |
CPDA-1 (Citrate*, phosphate, Dextrose, adenosine)
35 days ****platelets only 5 days so dont order unless give!! (plasma 1 yaer) |
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WHen to oder platelets?
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Thrombocytopeia
prevent bleeding Thrombopasthemia |
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When to order plasma?
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in coagulation disorders
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Dosage you give for RBC?
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give 10mL/kg= 10% rise in hct
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Platelet Dosage?
1 unit/10kg= |
17-50,000 increase
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Highest risk of blood transfusion?
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Hep B
1:63000 |
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Immediate Transfusion Reaction
What is it? Cause? Sx Tx |
Immediate hemolytic reaction caused usually by ABO incompatablity.
Symptoms include (Fever, chest pain, Dyspnea, hypotension, tachycardia) TX: STOP transfusion Maintain BP with sailine Monitor renal fxn Monitor for coag defets (DIC) send spec to lab |
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Most common transfusion reaction?
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Febrile nonhemolytic reaction
(the get a fever) Still stop the transfusion |
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Post transfusion Purpura mcc?
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Pregnant P1 negative lady
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Whats a normal Hb?
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12.5-14.5
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What moniters oxygen delivery?
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Kidneys and when it gets low
they secrete EPO |
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EPO function?
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Stimulates erythroblasts and proerythroblasts to proliferate and make erythrocytes
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Clinical Features of Fe deficiency Anemia
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Fatigue
Palor Pica |
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MCC of Fe deficiency anemia?
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Chronic blood loss
(usually gut or GU) |
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Things for the Fe Dificient anemic to avoid?
- - - |
Caffine (coffee and tee)
Avoid High Fiber Avoid Calcium intake with iron |
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Typical Fe deficiency anemia treatment prescription?
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FeSO4 325 po tid for 6 mo
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Oral Iron adverse effects?
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GI
-Dyspepsia -Bloating/Constipation -Nausea Temporary Dental Staining |
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Fe drug-drug interactions
- - |
Decreases Absorbtion of Floxins and Tetracyclins
Increased GI toxicity with bisphoshponates Decreased Fe absorbtion with GERD drugs |
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Hemosiderosis Causes
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Sickle cell
Thals. APlastic anemia Myelodysplastic states and more |
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Best way to tx iron overload?
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phlebotomy**
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Drugs you can use in iron overload?
- - |
**Phlebotomy still #1
Deferoxamine Deferasirox |
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Deferoxamine
A. MOA B. Administration C. AE D. Contrain? |
A. Binds free Fe (chealates) and enhances urinary exretion
B. IV or sub Q C. Adverse Effects Injection site pain, Ototoxic Ocular Pain Hypersensitivity and shock CONTRAIN in ANURIA (not pee peeing) |
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Deferasirox
A. MOA B. Other info |
A. oral Chelator but pretty much like Deferoxamine
B. More expensive -AE milder? |
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Drugs that can cause Folate deficiency?
- - |
Methotrexate
Trimethoprim |
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When is folate suplementation contraindicated ?
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B12 deficiency
(reverses anemia and Increases Neurologic problems.) Common just to give Folate and B12 at same time |
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Drug that can be given in B12 deficiency?
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Cyanocobalamin
-IV and Sub Q way cheaper than oral form. -Exreted through urine and bile *Life long |
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Epo alfa contraindications?
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Fe deficiency
Non Anemia Chemo with a curative intent HTN |
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Epo Alfa AE?
- - - - |
DVT
Decreased Survivership in certain cancers HTN CHF |
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cousin of epo alfa just with a longer half life?
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Darbepoietin
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Hydroxyurea is not safe in?
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Pregnant or lactating women
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Hydroxyurea MOA?
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Dihydrofolate Reductase inhibitor
WHich inhibits DNA synthesis (S-phase specific) |
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Hydroxyurea contraindications?
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Leukopenia
THrombocytopenia |
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Why is hydroxyurea used in sickle cell?
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Increases HbF
Inccrases deformability of sickled cells (flexible) Alters their adhesion ***therefore decreases PAINFUL VASO-OCCLUSIVE crisis and hospital admin |
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Hydroxyurea AE?
- - - |
Leukopenia
THrombocytia Teratogenic |
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What are the myeloproliferative disoders?
- - - - |
CML (white cells)(#2)
Polycythemia rubra Vera (red Cells)(#1) Essential Thrombocytosis (platelets)#3 Myelofibrosis with myeloid metaplasia (Stroma) (least common) |
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Patient comes in and says. "Doc my feet feel hot!" You note warm and red feet. She also says they are painful. You also note signs of Hemorrhage and thrombosis. What is this most likely?
What are some common labs for this? |
Essential Thrombocytosis
Pt will have elevated plateles over 600,000 50% Chance of having a JAK stat problem Mega thrombocytes on smear |
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TX of Essential Thrombocytosis?
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Asprin
Hydroxyurea (and two other drugs but not used much) Normalizing the platelet count DOES decrease risk of thrombosis contrary to Harrisons |
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Myelofibrosis Symptoms
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Asymptomatic
Splenomegaly Fatigue Fever Night sweats gout (MOST asymptomatic or vague) |
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Myelofibrosis Lab findings?
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Excess firbrous on bone marrow
Tear drop cells and nucleated RBC's Thrombocytosis Leukocytosis (will also see hepatospleenomegaly) |
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Dacrocyte
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tear drop cell
(Myelofibrosis) |
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Polycytehemia workup.
(1st make sure to asses plasma volume for spurious polycythemia) What to think when there is an Increased EPO? Normal/Low EPO? |
Increased then think Secondary Polycythemia
(Next step is appropriate vs. not appropriate) Normal/low epo? Polychytemia Rubra Vera |
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When you have polycythemia with increased EPO
A.)DDX with appropriately raised EPO? B.) DDX with an inapropriately Raised EPO? |
A. APPROPRIATE
-Pulmonary, Cardiac, or Hb abnormalities (Body says Im starving because of my poopy heart! or whatever so make epo for more blood!!!) B.)INNapropriate*** Renal Pharmacologic Tumor |
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Polycythtemia clinical manafestations?
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CNS sx
-HA, Dizzy, TInnitis, Stroke Cardiac -HTN, Tacy, Angina, CHF General Fatigue, nausea, Thrombosis |
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THrombosis in polycythemia can manafest as simply as?
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nose bleeding or gum bleeding
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52 yo wallstreet trarder worth 52 zillion dollars comes in to your office. He is complaining of fatigue, angina, and dizzyness. You rule out MI, and begin a hematologic workup. Your CBC shows polychthemia, however bieng the awesome doc you are, you also note the plasma volume is only 25% of the total blood volume. What do you think this is doc?
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Spurious Polycythemia
(THe dude is stressed, probably smokes, and drinks lots of caffeine. And when the market looses 2 points he freaks out and vomits and poops himself silly) Gaisbock's disease (stress polycythemia) |
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EPO fun facts
A.Is a____horomne poduced in kindey in response to hypoxia B. Its target site is____? |
A. Glycoprotien whose gene is on Chromasome 7
B. Jak-2 receptor in blast cells of bone marrow |
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Etiology of polycythemia rubra vera?
When to suspect? Treatment? |
A gain of funciton mutation in the JAK-2 receptor
(suspect when epo is low*) Tx: Is phlebotomy to a hct of 45% (usually weekly until normal hct then do once a month (500cc/time)) |
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Third Test in polycythemia workup?
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Get Puls Ox to see if <92% on room air
(this tells you the Kidney is sensing low O2 therefore you will kick up EPO, NORMAL APPROPRIATE response) (pulmonary, cardiac, hb problems) >92% then its abnormal (renal, pharm, or tumor) |
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Some meds that can cuase leukocytosis?
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Corticosteroids
Lithium Filgramistim (GCSF) Sargramistim(GMCSF) stimulates granulocyte and monocyte line so duh! |
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Bone Marrow causes of Leukopenia? (primary leukopenia)
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Myelodyplasia
Aplastic anemia B12 , and Folate deficiencies |
