Hematology Notes

Iron Deficiency Anemia Hereditary Spherocytosis

- Low level/Lack of iron - A deficiency of ankyrin mRNA leading to a reduced synthesis of
- MCV: <90, microcytic ankyrin which, in turn, underlies the decreased assembly of
- Iron helps on RBC maturation spectrin on the membrane.
- During adulthood: injuries, infection, diet - Assembly of spectrin is decreased → structure and shape of cell is
- Inadequate iron = inadequate absorption decreased → more fragile → rigid cell
- S/Sx: Fatigue, SOB/DOB, Dizziness (due to lack of O2), Brittleness - Cells are round like a sphere.
of hair/nails - Causes: splenomegaly → jaundice
- Tx: ↑ iron intake, IV/IM iron sucrose, Vitamins & Iron, Iron - S/Sx: Fatigue, DOB, enlargement of the abdomen, weight loss
supplements through IV/IM - Tx: BT, Splenectomy
- Nx Mgt: Less work, Bed pads, 02 support, Avoid massaging of
infected area, Health Ed. Aplastic Anemia
- Causes: Radiation, virus, drugs (chemotherapeutic & antibiotics),
Pernicious Anemia / B12 or Folic Acid Deficiency chemicals (Benzene)
- Autoimmune, Malabsorption - Destruction of bone marrow due to stem cell disorder →
- B12 Intrinsic Factor → blocked by antibodies → antibodies pancytopenia (anemia, thrombocytopenia, leukopenia)
attacked intrinsic factor (glycoprotein). → Malabsorption → Lack - S/Sx: Dizziness, fatigue, DOB, bruising (petechiae), restlessness,
of nutrients in the body risk for infection, bleeding
- RBC → immature, has less condensation - Tx: BT, Bone marrow transplant, 2PRBC 4h apart + 5 Packs platelet
- MCV: >90, macrocytic. concentrate
- S/Sx: Dyspnea, Headache, Dizziness, Splenomegaly, Jaundice, - Nx Mgt: BT (monitor q30mins), monitor for allergic reactions, O2
Color Red Beefy Tongue, Tingling sensation of the extremities, support, reverse isolation (PPE), avoid IM/SQ injections, keep
Altered behavior venipunctures minimal, (IV site to be rotated q72h), educate pt to
- Tx: IV/IM B12 and folic acid, supplements, ↑ B12 and folic acid avoid the usage of razors
intake - Therapeutic time: >3 weeks
- Nx Mgt: Bed rest, health education, ↑ B12 and folic acid food
intake, IV Therapy, avoid heat application (prone to Sickle Cell Anemia
bleeding/bruising/burn) - Life-long disorder, hereditary
- Normal Hgb bind: 2⍺ and 2𝛽
- RF: Mediterranean races
- The sickle mutation causes substitution of a valine for glutamic
acid as the seventh amino acid of the beta globin chain.
- Glutamic acid → mutated to valine → poorly soluble when
deoxygenated → sickle cell shape.
- Sickle shape RBC = low O2/ deoxygenated.
- RF: African-American races
- Vaso-occlusive crisis = pain
- Vaso-occlusive crisis → penile area site → prolonged erection &
pain.
- Tx: locally induced anticoagulants (penile area, cut tissue then
administer anticoagulants, allow bleeding to remove sickle cells)
- Vaso-occlusive crisis on spleen → splenomegaly → splenic crisis
→ s/sx: jaundice, pallor
- S/Sx: arterial hypertension, delayed growth (puberty), eye
problem
- Complication: risk for infection, stroke, multiple organ failure
(MOF)
- Tx: 1mg Folic acid daily for live (if forgone, vaso-occlusive crisis
occurs), for 5 year olds: Penicillin daily (until early adolescent),
pain relievers, ↑ IVF, BT, Bone Marrow Transplant, hydroxyurea
(chemotherapy med)
- Ns Mgt: O2 support, WOF DHN, avoid tight clothing (loose
clothing should be worn), wounds properly dressed (kept clean
and dry), avoid anticoagulants, avoid pt to exert effort, encourage
↑ intake of foods rich in folic acid, avoid extreme cold.
Thalassemia
- Hgb disorder
- RF: Mediterranean races
- ⍺ Thalassemia/Primary Thalassemia/ Thalassemia Minor: 1⍺ and
2𝛽
- S/sx: fatigue, DOB, pallor
- Tx: folic acid, if < normal Hgb levels BT
- Nx Mgt: Bed rest, O2 support, hydration, health teaching,
↑ folic acid intake
- 𝛽 Thalassemia/Primary Thalassemia/ Thalassemia Major: 2⍺ and
1𝛽
- Fragile RBC → shorter lifespan → severe hypoxia, skeletal
deformities, excess iron supply
- Excess iron supply → decrease function of different
organs
- S/Sx: poor appetite, lethargic, fatigue, pallor, jaundice,
splenomegaly, growth retardation
- Women: problem in menstruation/puberty
- Men: erectile dysfunction, fail to undergo puberty
- Tx: regular BT (q6mos/3mos), ↓ iron intake, chelation
therapy (remove metals like iron in body), splenectomy,
bone marrow transplant, emotional/physical support
- Complications: HF, pericarditis, lung punctured by ribs
(needs support always)
- Nx Mgt: O2 support, emotional/physical support, V/S
q1h, palliative care, deterioration
- MCV: >90, macrocytic (both)
Hemorrhage
- Excess bleeding → low RBC → low O2
- S/Sx: pallor, dizziness, DOB, fatigue
- Mx Mgt: wound care, dressing (keep clean and dry), BT
- Nx Mgt: WOF signs of bleeding, keep wound dry and clean,
monitor V/S
Polycythemia Vera
- First Degree/ Primary PV: Defect/overproduction of RBC in the
bone marrow
 - Tissue receptor in the bone marrow is hyperactive → - Hemophilia A: F-VIII deficiency/lack, 1 out of 5,000 males have it
increased EPO → increased RBC → increased viscosity → (aka Classic Hemophilia)
increased blood clot → emboli, thrombi → CVD, Stroke - Hemophilia B: F-IX deficiency/lack, 1 out of 30,000 (aka Christmas
- Second Degree/ Secondary PV: Defective EPO production Disease because considered rare)
- Kidneys secrete increased EPO → increased EPO → - Stages:
increased RBC → increased viscosity → increased blood - 1. Delayed Bleeding (Mild) - profuse bleeding after awhile
clot → emboli, thrombi → CVD, Stroke (1 to 2 minutes after injury)
- S/Sx: ↑ bleeding, CVD, ↑ renal cancer, hypoxia, purplish - 2. ‘Deep’ Bleeds (Moderate) - hemorrhage inside tissue
hue of skin, splenomegaly, hepatomegaly, abdominal (check for hematoma to assess)
pain/discomfort, tingling sensation in the extremities, - 3. Spontaneous Hemathorses (Severe) - bleeding tissue,
altered behavior, weakness bones from minimal movement.
- Mx Mgt: Phlebotomy (remove excess blood 250-500mL), - Tx: Desmopressin (Mild), Prophylaxis & Coagulants
hydration (fast drip IVF 60 gtt/min), Allopurinol reduces (Mild/Moderate/Severe)
high uric acid levels - Nx Mgt: WOF bleeding, immobilize, educate pt/watcher/parent
- Nx Mgt: assist during ambulation, safety, avoid hot how to control bleeding, soft-bristles toothbrush, avoid aspirin,
showers, tap water for showering, V/S monitoring q1h diet
- Pt education: rest, immobilize, cold compress (fast blood clotting),
Disseminated Intravascular Coagulation (DIC) elevate wounded/injured area, monitor for joint pains, don’t
- RF: Pregnancy, Cancer, Sepsis, BT reaction, Infection, Liver disease engage in any sports, regular dental check-ups
- 1. Clotting factors - ↑ clotting
- 2. Reduced BF to tissue and organs → MOF
- 3. No clotting factors left
- 4. Hemorrhage
- S/Sx: DOB, organ failure, low platelet count, bruising, ↑PTT time,
↑PT time, ↑ D-dimer
- Mx Mgt: Anticoagulants, IV antibiotics for infection, BT (platelet
concentrate), IVF
- Nx Mgt: WOF Bleeding, V/S monitoring, safety, hydration, provide
heplock, if venipuncture contraindicated use NGT for meds,
prevent further complications

Hemophilia
- Hereditary, inability/disorder in blood clotting. -

- 2) TTP: rare, life-threatening blood disorder. Blood clots form in
- -
Thrombocytopenia
- a condition in which the platelets (also called thrombocytes) are
low in number, which can result in bleeding problems.
- Normal platelet count: 150,000 to 450,000 platelets per microliter
of blood.
- Types: 1) Idiopathic Thrombocytopenic Purpura, 2) Thrombotic
Thrombocytopenic purpura
- 1) ITP: antibodies is attacking the platelets GIIb/IIIa receptors
which are responsible for platelet formation → destruction of
receptors → lowers immunity
- Occurs from childhood to early adulthood
- Platelet complex is too large to fit in BV to the spleen due
to antibodies attached to the GIIb/GIIIa receptors.
- Lesser platelet count → ↑ RBC production
- 2 Types of ITP
- A. Acute ITP: causes are cancer, virus, infection,
chemotherapy drugs.
- S/Sx: epistaxis, mucosal bleeding, purpuric rash,
bruising
- B. Chronic ITP: autoimmune
- S/Sx: epistaxis, mucosal bleeding, purpuric rash,
bruising and splenomegaly
- Tx (both): IV corticosteroids, IVIG, Anti-D antibody (fights
the stimulants of the production of high levels of
antibodies)
- Nx Mgt: WOF bleeding, prevent bruising (caution objects
and sharps), safety, reverse isolation (protect from
infection), avoid aspirin (blood thinner → risk for
bleeding)
small blood vessels throughout your body.
2 Types of TTP
- A. Familiar TTP/ cTTP (congenital)
- Hereditary or birth. Uncommon.
- Deficiency of ADAMTS13 at birth.
- Both parents have TTP genes (50-50, not x or y
bound)
- Same symptoms with iTTP.
- B. Immune-mediated TTP/ iTTP (immune) / aTTP
(acquired)
- Develops during late childhood to early
adulthood.
- Von willebrand factor: acts as glue
- ADAMTS13: turns off VWF
- Antibodies attack ADAMTS13 → enzyme is
destroyed → no VWF neutralization → low
platelet count in the circulation due to
uncontrolled blood clotting → increased
destruction of RBC or bleeding
- S/Sx: headache, mental changes, confusion,
speech abnormalities, paralysis, seizure, coma,
purpuric rash, fever, weakness, fatigue, extreme
 pallor, heavy bleeding, abdominal pain, nausea, - Conducting BT:
vomiting - 1. BT Consent - no sigh, no transfusion
- Complications: CVD, stroke, 10% of pts have - 2. Crossmatching result
kidney failure (needs dialysis) - 2. PNSS isotonic, G20 and above (18, 16, 14)
- Tx (both): plasmapheresis, steroids, rituximab (decrease - 3. Double checking - RN to RN, MT to RN (name, blood
production of antibody that attacks ADAMTS13) type, expiration date, serial number, *if anomaly is
- Nx Mgt: WOF bleeding, bruising, safety, physical and present, return to blood bank)
emotional support, protect pt from infection, monitor - 4. V/S monitoring: q15m first hour, q30m for whole
v/s. duration
- 5. Make sure BT line is patent
Blood Transfusion - 6. PNSS should be KVO (15 - 16 gtt/min)
- 7. If air bubble is present in BT line, stop immediately and
Compatible Incompatible Donate
take it out (from air bubble down to the end of line, drain
A A/O AB/B A/AB blood until air bubble is removed) emboli risk

B B/O AB/A B/AB

Infusion Temperature (for
AB A/B/AB/O — AB preserve)

O O A/B/AB A/B/AB/O PRBC 6h 4C

Washed RBC 2h 4C
- AB: universal recipient
- O: universal donor FFP (plasma) ASAP -18C
- Rh negative to Rh negative
Platelet ASAP -18C
- Rh positive to Rh positive and Rh negative
- Example: AB- can receive Rh+ and Rh- from everyone BUT WB 2h 4C
can only donate to Rh-.
- Blood Expiration:
- 1. Plasma - <1h
- 2. Platelets - 5 days if refrigerated
- 3. WBC/RBC - 5 to 7 days if refrigerated (6h in warm
temp)