Hemoc Flashcards
List the factor deficiencies and what type of bleeding do they present with?
Factor 8- hemophilia A
Factor 9- hemophilia B
Factor 11- Jews
Joint bleeding, retro peritoneal bleeding, cranial bleeding
What are the three branches of coagulation disorders?
Factor deficiencies, vWD, thrombocytopenia
Which is a platelet activity problem?
VonWildebrands deficiency
List the thrombocytopenias and the type of bleeding they cause.
TTP, ITP, DIC all cause mucous membrane bleeding. Gums, petechia, purpura, vaginal bleeding
Normal platelet count?
150,000-400,000
Lab test for factor VIII deficiency and treatment?
Joint bleeding, normal PT, in erases PTT, normal thrombin time. Dx by reduced factor 8 activity, treat with factor concentrate or FFP
Describe hemophilia B.
Factor IX deficiency, x linked, bleeding into joints etc, men and less common. Normal PT, increased PTT, reduced factor 9 activity. Treat with Factor IX concentrate or FFP.
Describe the classic scenario of Factor XI deficiency and treatment.
Ashkenazi Jew with post op bleeding. Labs show prolonged PTT and reduced levels of factor XI. Treat with FFP.
What type of problem is vonWillebrands disease?
Most common severe congenital bleeding disorder. Problem with platelet adhesion not numbers. Bleeding in nasal, sinus, vaginal and GI mucosa. Labs show normal platelet count, prolonged PTT, decreased vWF. Treated with vWF concentrate and vasopressin.
What is ITP?
Immune thrombocytopenic purpura
Describe ITP.
Most common thrombocytopenia in adults. Women with other autoimmune disorder and children after a viral infection common. Presents with petechiae and purpura and gingival bleeding. NO splenomegaly.
What causes ITP?
Igg attaches to platelets and when they get to the speen they are filtered, out by macrophages. Treat with prednisone, splenectomy, IV immunoglobin
Describe Thrombotic thrombocytopenic purpura.
Seen in previously healthy young women. Consumes platelets.
What are the five classic findings in TTP?
- Thrombocytopenia
- Hemolytic anemia with schistocytes
- Fever
- Neurological signs
- Renal involvement (hematuria)
What childhood disease resembles TTP?
HUS but in children there is no neuro involvement.
How do you treat TTP?
FFP and plasma exchange
Steroids and ASA
What is DIC and what causes it?
An acquired thrombohemorrhagic disorder…. From excess thrombin and plasmin.
Something has to trigger it!
What are some signs and symptoms of DIC?
Clotting and bleeding. Ischemia, coma, respiratory failure, ulcers, inter avascular hemolysis, fever, hypotension, petechiae, purpura, hematomas, massive bleeding, epistaxis, hematuria
How do you treat DIC?
Treat the underlying cause. Heparin therapy but remember to give FFP, and platelets.
What is HIT and treatment?
Heparin induced thrombocytopenia. Less common in LMWH but it happens. Patient produced antibodies against heparin compound and they the. Become sticky and clots form. Common to have thromboembolic events. Treatment is to STOP heparin!
Acute lymphocytic leukemia (ALL)… Describe it.
Most common in children around age 4. 70% cure rate. Patient presents with anemia, bruising, infection, cns involvement, lymphadenopathy and splenomegaly.
What are the labs for ALL and the treatment plan?
Over 30% blasts! an elevated white count! anemia! need bone marrow for dx. Treat with chemo- daunorubicin, vincristine, prednisone. Methotrexate for CNS treatment.
What is CLL and how do the patients present?
Chronic lymphocytic leukemia. Cause unknown and it is men over 50 with hx of recurrent infections. Mainly B cell lymphocytes. Clinically asymptomatic with splenomegaly and lymphadenopathy. Slow indolent course.
What do the labs and treatment look like for CLL?
Lymphocytosis, SMUDGE cells, treatment is chlorambucil to reduce white count and chemo-fludarabine.
Smudge cells?
CLL
What is AML and how do the patients present?
Acute myelogenous leukemia. More common in adults with a hx of radiation or hx of chemo. Poor prognosis. Patient. Presents with fatigue, anorexia, dyspnea, lymphadenopathy, splenomegaly.
What do the labs for AML look like and what is the treatment?
Blasts >30%, Auer rods, elevated white count! and need bone marrow for dx. Treat with chemo.
Auer rods?
AML
What is CML and how does the patient present?
Chronic myelogenous leukemia. Middle aged patient with fatigue, SOB, weight loss, easy brushing and splenomegaly. Lbs are positive for Philadelphia chromosome, anemia, elevated LDH, and CBC diff shows entire white cell line. Treat with imatinib.
What is hairy cell leukemia?
Common in men 55 years with a hx of frequent infections. Patient presents with pancytopenia and splenomegaly. Hairy cells on bone smear. Treat with 2-CdA.
Describe Hodgkin’s lymphoma and how the patient presents.
Two ages….. 15-35 and over 50. Possibly from EBV. Classic Reed-Sternberg cells on smear. Patient has painless lymphadenopathy, splenomegaly, Hepatomegaly, and B symptoms (fever, night sweats, weight loss). Labs for staging.
What are the stages for Hodgkin’s and non Hodgkin’s lymphoma?
Stage 1: single lymph node
Stage 2: two or more lymph nodes on same side of diaphragm
Stage 3: lymph nodes on both sides of diaphragm
Stage 4: involvement of other organs.
Hodgkin’s lymphoma treatment?
Chemotherapy depending on stage.
Describe non Hodgkin’s lymphoma.
More common, men presents with lymphadenopathy and splenomegaly and B symptoms. Can involve skin, GI, and CNS. Staging same at Hodgkin’s. Treat with chemo.
Reed-Sternberg cells?
Hodgkin’s lymphoma.
Describe multiple myeloma.
Plasma cell dyscrasia. Bence jones proteins in the urine. Increased risk with age and blacks with risk factors of radiation or asbestos exposure.
How do patients with MM present?
Early with fatigue, weakness, weight loss. Later with bone pain, anemia, renal function changes, neuro abnormalities. Skeletal pain on exam with bruising and wasting. Recurrent infections are common.
What are classic X-ray findings with MM?
Punched out lesions in skull, ribs, spine and pelvis.
Treatment for MM?
Supportive therapy, chemotherapy melphalan, prednisone.
Bence jones proteins?
MM
Rbc x 3= hgb
Hgb x 3= hct
True
What is the normal MCV of a RBC?
80-100
List the microcytic hypo chromic anemias. There are 5.
Iron deficiency anemia Thalassemia Anemia if chronic dz Lead poisoning Sideroblastic anemia
What is the formula for a corrects retic count?
Observed retic [hct/norm hct]= corrected retic
Less than 2%= decreased production
Greater than 2% = increases destruction
List causes of iron deficiency anemia.
GI blood loss Colon cancer Vaginal blood loss Urinary blood loss Malabsorption like crones Increased demand like pregnancy Poor intake like anorexia
Lab features of iron deficiency anemia.
Microcytic, hypochromic
Decreased serum iron
Increased TBIC
Decreased iron stores in bone marrow
How to treat iron def anemia?
Elemental iron 60 mg per day
Best test to determine response to iron therapy in iron def anemia?
Retic count
What is a thalassemia?
Problem with hemoglobin synthesis. Two types tha A and thal b.
What are the types of beta thalassemia?
Major and minor.
Describe beta thalassemia minor and the treatment.
Patient asymptomatic. Target cells on smear. Treatment is genetic counseling and to avoid iron.
Describe beta thalassemia major and treatment.
Signs begin at 6 months. Failure to thrive, hepatosplenomegaly, expansion of bone. Treatment is transfusions, splenectomy, iron chelation.
Describe the 4 states of alpha thalassemia.
Silent carrier- 1 gene inactive: no sx.
Alpha thalassemia trait- 2 genes. Mild anemia, no sx.
Hemoglobin h dz- 3 genes, anemia, splenomegaly, hgb h 4-10%
Hydrous fetalis- 4 genes, death in utero
Three Meds that cause sideroblastic anemia?
Chloramphenicol, INH, alcohol
Too much iron so elevated ferritin and iron, low TIBC
Treat symptomatically or use pyridoxine (vit b6)
Describe the s/s of lead poisoning.
Abdominal pain, constipation, neurological changes, intellect changes
Anemia with basophillic stippling?
Lead poisoning!
Treatment for lead poisoning?
Remove from environment, chelation
Causes of microcytic anemia?
Nutrition liver dz, prelukemia, drugs like methotrexate and phenytoin
What can lead to decrease V B12 absorption?
Crones dz because it is absorbed in the terminal ilium, diet is only source.
Signs and symptoms of B12 deficiency and treatment?
Tingling in feet, gait abnormalities, gloss it’s, loss of vibratory sense.
Has hyper segmented neutrophils and vb12 level is low. Treat with IM injections.
Describe folate deficiency s/s and treatment.
Found I green leafys and absorbed in the proximal jejunim, 4 month stores common in alcoholics. Glossitis, and angular cheilosis. NO neurological sx. Treat with oral supplements.
How do you tell the difference between iron deficiency anemia and anemia of chronic disease?
Look at the ferritin. It is normal or elevated in AofCD and decreased in IDA.
What does the Coombs test show?
It tests for autoimmune hemolytic anemias.
Describe hyperspleenism.
Coombs negative hemolytic anemia. Increased removal of cells by the spleen. Secondary from infection, inflammatory dz, myeloproliferative disorder, leukemia and lymphoma.
Describe microangiopathic hemolytic anemia.
Coombs negative. SCHISTOCYTES, etiology HUS, DIC, TTP, prosthetic heart valves.
What is the number one cause in the world for microangiopathic hemolytic anemia?
Malaria
List the three types of Coombs positive hemolytic anemias.
Drug induced, warm antibodies, cold antibodies
Drug induced hemolytic anemia types?
Hapten- penicillins
Innocent bystander-quinidine, hctz, sulfonamides, rifampin
Autoantibody type- aldomet, ibuprofen, diclofenac
All tx is stop med!
Describe warm antibody Coombs positive hemolytic anemia.
IgG type. Causes are idiopathic, autoimmune (SLE, lupus)
Treatment is steroids, IV gamma globulin, splenectomy
Describe cold antibody Coombs positive hemolytic anemia.
IgM, triggers compliment. Causes are viral, lymphoma
Treatment:jeep patient warm, immunosuppressive but poor response to steroids
May have raynauds
Describe hereditary spherocytosis and the tx.
Many spherocytes on blood smear, autosominal dominant, common in Northern Europeans, cells become spheres and rigid.
Splenomegaly on exam. Treatment is splenectomy and folic acid
Describe hereditary elliptocytosis and treatment.
Autosomal dominant. Patients usually asymptomatic. Treatment is splenectomy which stops hemolysis but does not cure.
Describe G6PD deficiency and what things to avoid.
Sex linked in Mediterranean, west African, middle eastern, Southeast Asia. Stress leads to hemolysis. ANTIMALARIALS, SULFONAMIDES, favs beans, infections, DKA. Asymptomatic until crisis. Smear shows burr cells and Heinz bodies. Avoid triggers.
An 8-10 year old presents to clinic with complaints of RUQ pain after eating and has black gall stones and splenomegaly. What is the dx?
Pyre ate kinase deficiency.
What causes sickle cell anemia?
Substitution of valine for glutamine at position 6 on beta chain. This makes hgb s. Hgb s is poorly soluble at low oxygen levels.
What are some clinical features of sickle cell crisis?
Bone, chest and abdominal pain, splenomegaly, leg ulcers, pulmonary infarction, stroke. Lab test is hemoglobin electrophoresis.
Describe sickle trait.
Patients not anemic and rarely have sx. 25-45% Hgb s. Normal lifespan.
What is hemoglobin C disease?
Caused by hemoglobin crystals. Mild anemia, splenomegaly and target cells. Dx with electrophoresis.
Describe aplastic anemia and the treatment.
Decrease in production of all cell lines. Caused by NSAIDs, chloramphenicol, CMV parvovirus B19. Pancytopenia on lab. Treat with bone marrow transplant.
Anemia in men is HgB less than?
14
Anemia in women is hGb less than…
12
List the microcytic hypochromic anemias. Small pale!
Iron deficiency, thalassemias, lead poisoning, sideroblastic.
List the macrocytic anemias. BIG!
Vitamin B 12 and folate deficiency
Corrected retic less than 2?
Decreased production
Corrected retic count greater than 2?
Increased destruction.
What is the storage form of iron?
Ferritin
Best test to monitor iron therapy in iron def anemia patients?
Retic count!
Which anemias has target cells?
Thalassemias
What is a ringed sideroblast?
Where iron accumulates in the mitochondria forming a ring
In sideroblastic anemia there is a problem with the use of the iron… What is the treatment?
Give vitaminB6. Pyridoxine
Basophillic stippling?
Lead poisoning.
Pernicious anemia?
You need intrinsic factor to bind with vitamin B12 so you can absorb it. Low intrinsic factor is pernicious anemia.
Macrocytic anemia with neurological sx? (Glossitis, loss of vibratory sense)
Vitamin b12 def
Macrocytic anemia without neuro sx?
Folate deficiency
How to tell the difference between iron def anemia and AoCD?
Check ferritin level. It is an acute phase reactant so it will be LOW in iron def and normal or high in ACD.
Coombs test positive hemolytic anemia means what?
Not autoimmune. Something outside the cell is causing destruction.
Most common hemolytic anemia in the world?
Malaria
Schistocytes =
Microangiopathic hemolytic anemia. ( Coombs neg)
Three types of drug induced hemolytic anemia?
Hapten, immune complex, autoantibody.
Coombs positive hemolytic anemia warm typed caused by?
IgG. Treat with steroids. Warm, IgG, treat with steroids.
Coombs positive hemolytic anemia cold…..
Cold, IgM, immunosupression. No steroids!
Hereditary spherocytes is means what deficiency?
Spectrin, presents with splenomegaly. Treat with splenectomy and folic acid.
MC drug reactions in G6PD?
ANTIMALARIALS, and sulfonamides.
Burr cell=
G6PD deficiency.
10 your old child presents to clinic with gallbladder dz sx and black gall stones? Dx?
Pyruvsre kinase deficiency.
Sickle cell cause?
Sub of valine for glutamic acid at 6 on beta chain
Screening tests for sickle cell?
Sickledex.
Test of choice in sickle cell?
Hgb electrophoresis.
Cause of ITP in adults?
IgG binds to platelet which then gets phagosatised in the spleen
TTP….. Young healthy women. Must have these 5 things:
Thrombocytopenia. Microangiopathic hemolytic anemia (schistocytes) Fever Neuro signs Renal involvement
TTP is like what in kIDS.
HUS. But HUS has no neuro signs and more renal involvement.
What is a common cause of HUS in kids?
Hemorrhagic colitis from e. Coli or shigella.
What is the basis behind DIC?
Excessive thrombin ( causing clotting) Excessive plasmin (causing bleeding). WTF?
Common cancer that presents initially with DIC?
Acute promyelocytic leukemia
Scenario where it could be liver failure or DIC check Ddimer.
D dimer is positive in DIC.
Auer rods?
AML
Warfarin od or rat poison od?
Give vitamin k or FFP.
Where does warfarin work?
Vitamin k dependent factors in the extrinsic side so PT is affected.
What do you give for heparin od?
Protamine
Heat side of the coagulation cascade does heparin work?
Intrinsic. Longer PTT.
Aplastic crisis in sickle cell caused by?
Parvovirus b 19 in 70% of cases.
