Hematology Flashcards
There are few physiologic disturbances until hemoglobin reaches ≤ _____.
7-8 g/dL
Some signs and symptoms of anemia include:
Pallor, loss of palmar crease pigmentation, irritability, fatigue, exertional dyspnea, orthopnea, pica (iron deficiency), tachycardia, palpitations, headache, vertigo
_____ is the most important lab clue for the cause of anemia.
Reticulocyte count
- Can also examine a peripheral blood smear and…
- *Urinalysis, direct Coombs test, stool guaiac
An acquired failure of the hematopoietic stem cells that results in pancytopenia is known as _____ anemia.
Aplastic
- Most often idiopathic in developed countries
- *May be induced by certain drugs or infections → hepatitis and mononucleosis
How are severe cases of aplastic anemia treated? Mild cases?
- Severe- stem cell transplantation
2. Mild- Supportive treatment with transfusion of PRBCs and platelets
When CBC shows hemoglobin decreased from baseline, with markedly increased reticulocytosis you are dealing with _____ anemia.
Hemolytic
Bacterial and viral infections in children with sickle cell anemia can precipitate rapid _____, with sudden-onset of jaundice and pallor
Hemolysis
*Other causes- TTP, HUS, giant hemangioma, artificial heart valves, sepsis, DIC, Sickle Cell Anemia/Crisis
How do you treat hemolytic anemia?
Treat underlying infection!
Sickle cell disease is an autosomal _____ (recessive/dominant) genetic disorder of HgbSS. Affects 0.15% of African Americans.
Recessive
Sickle cell trait confers some resistance to ______ (specific disease).
Malaria
- individuals with trait may have episodic hematuria and inability to concentrate urine
- *affects about 8% of African Americans
Sickle cell disease can lead to ______ anemia because sickled cells are destroyed by the spleen.
Hemolytic
_____ is one of the first signs of hemolytic anemia, seen around 6-9 months.
Dactylitis- digit swelling. Also have delayed growth and development.
Osteomyelitis is a common infxn associated with ____.
Sickle cell disease
Vasoocclusion in spleen & RBC pooling leading to acute splenomegaly and rapidly decreasing hemoglobin is known as ______.
Splenic sequestration crisis
*Can lead to splenic infarction and functional asplenia
Some triggers of a sickle cell crisis include:
Cold weather, hypoxia, infxn, dehydration, ETOH, pregnancy.
Acute chest syndrome, back, abdominal, and bone pain are associated with a ____ _____ _____.
Sickle cell crisis
- Priapism is also common :(
- *Those with the trait may suffer from: pulmonary HTN, CHF, symptoms of fatigue, SOB due to decreased O2 affinity of HgbS
How is sickle cell disease diagnosed?
- CBC with peripheral blood smear:
DECREASED hemoglobin
DECREASED hematocrit
INCREASED reticulocytes
Management of sickle cell disease:
- IV hydration and Oxygen 1st step!!! Then narcotics for adequate pain control (AVOID Meperidine–> high doses can lead to seizures)
- Hydroxyurea: reduces frequency of pain crisis
- Folic Acid: needed for RBC production and DNA synthesis
- RBC transfusion therapy- only in a severe crisis
Children with sickle cell disease should be immunized for:
*Hint- SHiN
S. penumo
H. influenzae type B
N meningococcus
*should also receive prophylactic PCN from 4 months- 6 years.
Von Willebrand disease, Hemophilia A, & Hemophilia B are examples of _______.
Clotting factor disorders
______ is the MC inherited bleeding disorder.
Von Willebrand Disease
*vWF is necessary to anchor platelets to the injured vessel wall
Treatment of von willebrand includes:
DDAVP!!!!
____ is the MC acquired coagulopathy.
Vitamin K deficiency
- Many coag factors depend on Vitamin K
- *Neonates should receive a dose at birth
Vitamin K deficiency often occurs because of malabsorption, seen especially in patients with _____ (particular disease state).
Cystic fibrosis
Overdose of ______ (certain drug) can cause severe Vitamin K deficiency.
Warfarin
Deficiencies in Vitamin K may be secondary to:
Poor diet, liver failure, malabsorption, malnutrition, and the use of some drugs (especially broad-spectrum abx)
Hemophilia A is a result of factor ___ deficiency.
8
- X-linked recessive
- *Prolonged PTT but PT is normal
Hemophilia B is due to a deficiency in factor ___.
9
- Christmas disease
- *Indistinguishable from Hemophilia A clinically
Heparin overdose antidote is ____.
Protamine
Warfarin overdose antidote is ____.
Vitamin K
The most common hereditary hypercoaguable state is ______. Causes an increase in DVTs and PEs, especially in young patients.
Factor V Leiden
*Management- for high risk you need indefinite anti-coagulation. For moderate risk then prophylaxis during high-risk procedures
A reduced level of inhibitors of the coagulation cascade is seen with Protein ___ and ____ Deficiencies.
C & S
- C&S are natural anti-coagulants
- *Treatment- oral anticoagulation for life
Antithrombin III can be depleted in what conditions?
Pregnancy, liver disease, nephrotic syndrome, DIC
*Treated with anticoagulants
Hormonal Hypercoaguable states include-
- Pregnancy (especially in 3rd trimester and up to 6 weeks postpartum)
- OCPs (especially when smoking)
____ is a disorder that manifests clinically as recurrent venous or arterial thrombosis and/or fetal loss. Often occurs in association with SLE or other autoimmune disorder.
Antiphospholipid Syndrome
Signs and symptoms of antiphospholipid syndrome include:
o Recurrent arterial or venous thromboses: DVTs or PEs
o Autoimmune diseases
o Thromboses after surgical procedures
o 2nd trimester miscarriages/Pre-eclampsia
o Migraines
o Thrombocytopenia
o Valvular heart disease → heart murmurs
o Limb asymmetry
o Livedo reticularis →vascular condition characterized by purplish discoloration of the skin
Indefinite ____ therapy is often used for individuals with antiphospholipid syndrome.
Warfarin
Platelet counts less than 150,000, a common cause of abnormal bleeding defines _____.
Thrombocytopenia
- TTP is an example–> Thrombotic Thrombocytopenic Purpura (also ITP, NATP, and TAR)
- *HIT–> Heparin Induced thrombocytopenia (seen in hospitalized patients)
Treatment for ITP is rarely indicated. If it is it involves:
Corticosteroids, IVIG, anti-D immunoglobulin
*Avoid NSAIDs
____ is found primarily in children, particularly after infection with E. coli.
Hemolytic Uremic Syndrome (HUS)
- pregnancy and estrogen use may precipitate it in adults
- *treat in kids- conservative, fluids and electrolyte balance
- **treat in adults- plasmapheresis
Some clinical findings of TTP include:
- jaundiced or pale
- purpura, petechiae, pallor, abdominal pain, fever
- CNS sx- wax and wane over mins
- may have significant renal dz
- possibly pancreatitis
____ is a severe illness that alters the homeostasis between hemorrhage and thrombosis. There is activation of both coagulation (thrombin) and fibrinolysis (plasmin).
Desseminated Intravascular Coagulation (DIC)
Acute and chronic conditions associated with DIC:
Sepsis, burns, tissue injury, obstetric complications, trauma, asphyxia, malignancy, cirrhosis, severe transfusion reactions
Clinical findings with DIC…
- Skin and mucous membrane bleeding (particularly at puncture/wound sites) and shock are more common
- Thrombosis (commonly digital ischemia and gangrene) less often predominates
______ is associated with unrestrained growth of leukocytes and leukocyte precursors in the tissues
Acute leukemia
- 30-40% of childhood cancer
- *Hallmark- pancytopenia with circulating blasts
___ is the MC type of acute leukemia.
ALL- Acute Lymphoblastic Leukemia (80%)
*AML (Acute Myeloid Leukemia) is primarily a disease of adulthood
ALL has a peak incidence around 2-5 years and is MC in ____ (boys/girls).
Boys
____ (B/T) lineage is the more favorable type of ALL.
B
Clinical findings of ALL include:
- Fever
- Pallor
- Petechiae or ecchymoses
- Gingival bleeding / epistaxis / menorrhagia may be the presenting complaint
- Lethargy / malaise
- Anorexia
- Bone or joint pain
ALL is confirmed with ____ on the peripheral smear.
Blasts, bone marrow aspirate, or both
*Bone marrow biopsy should be done urgently!!
High risk of tumor lysis syndrome associate with ALL
-hyperuricemia, hyperphosphatemia, hyperkalemia
Bone pain, often in the spine, ribs, or proximal long bones is associated with ______ (type of cancer).
Multiple Myeloma
A HIGH Transferrin and HIGH TIBC level suggests ____ anemia.
Iron deficiency
*With low Ferritin
A LOW Transferrin and a LOW TIBC is associated with _____.
Anemia of chronic disease
*With high Ferritin
A microcytic anemia w/ normal or increased serum Fe or no response to Fe treatment is known as _____.
Thalassemia
_____ is EPISODIC hemolytic anemia associated with sulfa drugs, fava beans, and infections.
G6PD deficiency
HUS has a higher associated with kidney involvement than TTP and does not classically have fever or ______.
Neuro sx
The 1st step in the workup of anemia is a ____ count.
Retic
- High retic= brisk bone marrow response to hemolysis or blood loss
- *Low retic= deficient RBC production
Low retic count can be broken down to: Normocytic (80-100), Microcytic (<80), or Macrocytic (>100).
- Microcytic Anemia- Fe deficiency, lead, thalassemia, early anemia of chronic dz.
- Normocytic Anemia- Anemia of chronic disease!, early iron deficiency
- Macrocytic Anemia- B12 deficiency, Folate deficiency (also liver dz, ETOH, HYPOthyroidism)
Celiac disease and Crohns affect the ileum (and absorption) and therefore can lead to malabsorption and _____ deficiency.
B12
Autoimmune destruction/loss of gastric parietal cells that secrete Intrinsic Factor is the defining characteristic of _____ anemia.
Pernicious
Clinical manifestations of B12 deficiency include:
Paresthesias, gait abnormalities, memory loss, and dementia
GI- anorexia, diarrhea, glossitis
B12 defiency should be treated with _____. Watch for signs of HYPO____ with treatment.
IM B12
Watch for HYPOKALEMIA
Common etiologies of iron deficiency anemia include:
Excessive menstruation, occult blood loss (colon cancer, parasitic hookworms)
Pagophagia (ice craving), pica, angular cheilitis, and koilonychia (nail spooning) are associated with ____ anemia.
Iron deficiency
Management of iron deficiency anemia includes:
Iron replacement (on empty stomach)
*Vitamin C increases Fe absorption
Acquired sideroblastic anemia that is MC in children is known as _____.
Lead poisoning anemia
Think THALASSEMIA if microcytic anemia with NORMAL/HIGH SERUM FE or no response to Fe treatment
fyi
Heinz bodies are associated with ______ deficiency.
G6PD
_____ is a lymphocyte neoplasm that is bimodal (peaks at 20 then again >50y) and associated with EBV.
Hodgkin’s Lymphoma
Clinical manifestations of Hodgkin’s Lymphoma include:
- Painless lymphadenopathy. Alcohol may induce node pain!
2. Systemic “B” symptoms: fever, night sweats, and weight loss
Reed-Sternberg cell is associated with a diagnosis of _____ (Hodgkin/Non Hodgkin) Lymphoma.
Hodgkin
*Also, mediastinal LAD on PET/CT scan
How is Hodgkin Lymphoma treated?
- Stage I, II, and IIIA- radiation therapy
- Stage IIIB, IV- combo chemo
* Highly curable- “Good” Lymphoma
____ is MC in individuals >50y. Peripheral LAD!! (Hodgkin/Non Hodgkin)
Non Hodgkin
*RF- Increased age, Immunosuppression (HIV)
An overproduction of all 3 Myeloid cell lines (primarily RBCs) is known as ______.
Polycythemia
- Peaks 50-60y, MC in men
- *Caused by JAK2 mutation
Clinical manifestations of polycythemia include:
HA, dizziness, tinnitus, PRURITUS (esp after hot bath), fatigue and thrombosis
*PE: Splenomegaly, flushed face
**Due to increased RBC mass
How is polycythemia managed?
Phlebotomy! –> Done until hematocrit <45%
Hydroxyurea
