1. The document provides information on various hematological conditions including indications for blood transfusion, iron and vitamin deficiencies, hemolytic anemias, leukemias, lymphomas, and bleeding disorders.
2. Key points covered include diagnostic criteria and testing, typical clinical presentations, complications, and treatment approaches for conditions like iron deficiency anemia, thalassemia, vitamin B12 and folate deficiency, hemolytic anemias, sickle cell disease, leukemias, lymphomas, immune thrombocytopenia, hemophilia, and von Willebrand disease.
3. Treatment recommendations are provided for issues such as indications for blood transfusion in sickle cell disease, chemotherapy regimens for
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Hematology Summary Guide
1. Hematology summary
Indications of blood transfusion in patient with anemia:
1. Healthy young patient HCT 20%
2. Old patient with comorbidities HCT 30%
Note: every I unit of PRBC increase HCT by 3%
What is daily iron requirements?
Woman 2-3 mg/day
Man 1 mg/day
What is the maximum iron absorption capacity?
3-4 mg/day
What is the indication of bone marrow biopsy in IDA?
IDA on blood tests, but can’t find the bleeding source.
What is the cause of death in untreated thalassemia major?
CHF
When does anemia become symptomatic?
HCT 30% fatigue and exercise intolerance
HCT 25% tachypnea and dyspnea
HCT 15-20% CNS symptoms
What should you always remember about anemia of chronic disease?
There’s anemia of chronic disease and renal failure anemia
What is the difference between howel jolly bodies and pappinhemir bodies?
Howel jolly bodies Basopilic stippling Pappinhemir bodies
DNA Ribosomes Iron overload
Single peripheral Multiple peripheral Many variable
Splenectomy IDA, thalassemia, lead poisoning Sideroblastic anemia
What are causes of sideroblastic anemia?
Congenital aminolevulinic acid synthase or B6 metabolism
Aquiredchloramphinicol, INH, alcohol, lead toxicity, myelodysplasia, CTDmyelodysplasiaAML
What are the complications of thalassemia?
Chronic anemia & CHF. Transfusion dependence; cirrhosis & hemochromatosis.
Large spleen, bone deformities.
Beta thalassemia: Alpha thalassemia:
Silent carrier or trait or minor B(B+/B0) Silent carrier
Disease intermedia B+(B+/B0) or major (B0/B0) trait
2. Disease
1 gene deleted 2 genes deleted 3 genes deleted
Alpha thal Normal clinical & labs Mild microcytic anemia; HCT 30-40 Moderate anemia; HCT 20-30
Beta thal Normal clincal & labs Severe microcytic anemia st 6 months with growth failure --
What parasite causes B12 defeciency?
Tapewarm; diphyllobothrium latum
What are the causes of folate deficiency?
Eczema, dialysis, anticonvulsants, alcohol.
How long does the liver store B12 and folate?
B12 3 yrs, folate 3 months
At what levels of the spinal cord does B12 damage?
Posterior and lateral spinocortical, and spinocerebellar. But can cause anything.
Note: methylmalonate & homocystine elevated in B12 def. only homocystine elevated in folate def.
What are the causes of hemolytic anemia?
Acute medication, autoimmune, G6PD
Chronic RBC abnormalities, PNH
Anemia Picture Diagnosis Treatment
IDA Glossitis, brittle nails, poikilonychia, pica
Iron studies; blood smear
Bone marrow biopsy
Oral ferrous sulfate
Parenteral; Best: BT (not used)
Chronic dx Same as any anemia Iron studies
Iron and erythropiotin only if renal
dx or cancer Tx
sideroblastic Same as any anemia
Iron studies, prussian blue; pappinhemir
bodies, BM RE iron high
Tx cause, B6 2-4 mg, iron chelation
Thalassemia Same as any anemia
Iron studies, electropheresis: ↑H, A2 in
beta thalassemia, target cells. Crew-cut
on skull xray
B major BT 1-2/month.
Splenectomy +/- BMT
B12 defect Neurologic signs, glossitis, diarrhea, abd pain
Blood smear, ↓retic, hypercellular BM
B12 and methylmallonate & homocystine
levels. Schilling test
B12 + Folate therapy; watch for
HYPOKALEMIA
Folate defect Same as any anemia Same as B12 defeciency Folate replacement
Hemolysis
Same as anemia, jaundice & dark urine
Rapid fever, chills, chest & back pain, ↑HR
Labs, blood smear, urine has hemosiderin
not bilirubin
Hydration to protect kidneys
3. Sickle cell
Painful ↓O2, dehydration, ↓pH, fever,
infection
Aplasticspleen sequester, G6PD, B19, folate
def
Hemolytic, Vasoocclusive, sequestration.
Affects all symptoms
↑retic unless folate defec/B19
Electropheresis, blood in urine
↑WBC, sickle prep
Fluid, analgesics, O2, ceftriaxone
Severe BT / exchange transfusion
Folate + vaccine pneumo+influenza
Prophylactic penicillin 4 mnth -6 yrs.
Hydroxyurea.
Autoimmune Fever, syncope, CHF, hemoglobinuria, cyanosis Coombs, cold agglutinin, spherocyte
Steroid splenectomy
immunomodulators
If CD20 rituximab
Spherocytosi
s
Splenomegaly, cholecystitis in young Osmotic fragility test Splenectomy
PNH Venous thrombosis (budd-chiari); early morning
Sugar-water &Ham test, ↓DAF
Most accurate flow cytometry for
CD55,59
Iron, steroid, anticoagulation,
eculizumab, BMT
G6PD Jaundice, dark urine, weakness, tachycardia Heinz bodies; bite cells. G6PD level Hydration and transfusion
Aplastic
anemia
Pancytopenia Pancytopenia+empty bone marrow
BMT, if not possible
immunosupressio
antithymocyte
globn+sterod+cyclosporin
Aplastic crisis Blood transfusion Recovery in a week
Painful crisis Self-limited Recovery in a week
Dactylitis in children -- --
Acute chest sx -- --
Splenic infarcts Splenomegaly in childhood. Not palbable at 4 yrs
Avascular necrosis -- --
Priapism Nifidipine/hydralazine Self limited erection for 30 min to 3 hr after cold, exercise, void
Sustained priapism Medical ER Erection > 3 hrs
Papillary necrosis Sel-limited --
What are the causes of autoimmune hemolytic anemia?
1. Blooc cancer lymphoma, CLL, leukemia
2. Viral infections
3. CTDs
4. Medications antibiotics, thiazide, methyldopa
5. UC
Which type of AIHA is more common?
Warm >> cold
Warm AIHA Cold AIHA
medications, CTDs, cancer (lympphoma, CLL) Mycoplasma & IM
Intravascular Extravascular
Splenomegaly Liver
IgG IgM
4. Direct coombs +ve & spherocytes Complement-coated RBCs OR cold agglutinin titer
Steroid & splenectomy Avoid cold & chemotherapy
What are the causes of cold-agglutnin hemolytic anemia?
1. Mycoplasma, mononucleosis
2. Lymphoma, waldenstorm macroglubinemia
What are the complications of PNH?
Aplastic anemia and leukemia
What is the cause of death in PNH?
Budd-chiari syndrome
What are the excerbation factors of G6PD?
Infections, medications (sulfa, nitrofurantoin, malaria, dapsone), fava beans
What is the typical picture of aplastic anemia?
Pancytopenia, empty bone marrow
What is the benefit of hydroxyurea in SCD?
Reduce recurrence of painful crises, and accelerated leg ulcers healing
What are the indications of blood transfusion in SCD?
CNS, LUNG, HEART, PRIAPISM
What are the causes of spherocytosis?
Congenital, G6PD, fever, autoimmune hemolytic anemia, ABO incompatibility
What are the causes of aplastic anemia?
1. Radiation and chemotherapy
2. Benzene
3. NSAIDs, chloramphinocol
4. Infections CMV, EBV, B19, CMV, hepatits, TB
5. Cancer infiltration
What are the causes of acute leukemia?
1. Aplastic anemia
2. Down and klenfilter syndrome
3. Myleodysplasia
4. Sideroblastic anemia
5. Retroviruses
What is the pathogenesis of acute leukemia?
Normal blood cells number, non functional (functional pancytopenia)
What translocation is associated with APL?
t(15:17) pancytopenia start ATRA immidiately (very sick patient)
What are the poor prognostic factors in ALL?
2<age<9. WBC>10^5. CCNS involvement.
5. ALL AML
Children Adults
Can’t ddx clinically
Extramedullary infiltration Extramedullary infiltration
Leucostasis causes vasocclusion pheresis+chemo Leucostasis causes vasocclusion
Pancytopenia Pancytopenia
Leukemic blasts on smear Leukemic blasts on smear
BM > 20% blasts BM > 20% blasts
CALLA, TdT Auer bodies, myeloperoxidase, esterase
Remission induction consolidation
Relapse BMT (the leukemia most responsive to Tx)
Remission induction consolidation
Relapse BMT
Cystosine arabinoside+daunorubicin/idarubicin
Daunorubicin, vincristine, prednisone
Intrathecal methotrexate to CNS prevention
Testicular disease Skin nodules
CML CLL
Functional myeloid cells Mature B cells
Philadelphia chromosome CD19; smudge cells
neutrophilia (left shift) + B symptom + massive splenomegaly
+ bone
No LN, no bleeding or infection
Lymphocytosis/LN/splenomegaly/anemia/↑platelets
No bleeding or infections; autoimmune hemolysis
Leukocostasis 200-500.000 vaso-occlusion --
Imatinib
Stage 0-1 nothing
Stage 3-4 fludarabine +/- rituximab ITP/hemolysis
Basophilia is present in all myeloproliferative disorders
What chromosome is associated with MDS?
5q
What is MDS picture?
Pancytopenia + blasts 1-20%
Leads to AML
Bi-lobed neutrophil (pulger-heut)
Tx: peroidic transfusion
TNF inhibitors: lenalidomide/thalidomide
5q: azacitidine/decitabine
Young patient BMT
What is multiple myeloma picture?
Picture Bone pain + infections + renal failure
Hypercalcemia + anemia + leucostasis
Diagnosis protein electropheresis + skeletal xray + B2 microglobulin + calcium level + KFT +
urinanalysis in acid urine + bone marrow biopsy (>10% plasma cells)
6. ** note: albumin level is low in multiple myeloma
Criteria: plasma cells > 10% + (M protein in plasma/urine/lytic bone lesions)
Tx
a. <70 yrs BMT + thalidomide + dexamethasone (DON’T GIVE CHEMO TX if considering BMT)
b. > 70 yrs mephalan + prednisone
c. Hypercalcemia fluid + duiretics + bisphosphonates
DDx : MGUS bone marrow plasma cells < 10%
protein electopheresis IgG spike <3 g
bence jones protein < 1 g/24 hr
no end-organ damage
What is the most common cause of death in MM?
Infections (lung or UTI)
What complication of MM is a medical emergency?
Cord compression by fracture or plasmacytoma immidiate steroid
MM CRAB Skeletal xray+plasma cells>10%+bencejones+electropheresis IgG>3 BMT/chemo+steroid
Waldenstorm LN/splenomegaly/hyperviscosity/neuro No bone lesions+IgM>5+bence jones Chemo+plasmapheresis
MGUS Asymptomatic plasmacytosis No end-organ+bence<1g/d+IgG<3+plasma cells<10 --
Hodgkin’s Non-hodgkin’s
Cancer of lymphocytes in LN; centripetal Cancer of lymphocytes in LN and extranodal
Reed-sternberg No reed-sternberg
Idiopathic Infections; HIV, EBV, HTLV-1, Hep C, h.pylori +immunosupression+CTDs
Palbable LN + B symtoms + Extranodal + pruritis Palbable LN + B symtoms + Extranodal + pruritis
-- HIV, EBV burkitt, HIV immunoblastic lymphoma + CNS involvment
Excisional biopsy + CT for staging + BM biopsy Same (BM biopsy is more important than in HL)
Stage 1 & 2 radiation
Stage 3 & 4 OR B symptoms ABVD >> MOPP
Stage 1 & 2 radiation
Stage 3 & 4 OR B symptoms R-CHOP (1st
test for hep B & C)
CNS radiation + CHOP
Multiple myeloma Pancytopenia No LN No splenomegaly
Hodgkin No pancytopenia LN Splenomegaly
NHL Pancytopenia LN Splenomegaly
Acute leukemia Pancytopenia LN Splenomegaly
CML Pancytopenia LN Splenomegaly
CLL Pancytopenia LN Splenomegaly
What are the causes of ITP?
1. Blood cancer Lymphoma & CLL
2. Infections HIV
7. 3. CTDs
How is ITP diagnosed?
1. Thrombocytopenia
2. Antiplatelet antibodies
3. Bone marrow full of platelets & exclude other causes
What is the treatment of ITP?
Prednisone splenectomy thrombopoietin agents ( romiplostim/eltrombopag)
PLT < 10.000 IVIG in Rh +ve patients
When type of thrombi are found in TTP?
Hyaline thrombi of smallest vessels fatal if not plasmapheresis done
What is the main complication of HIT?
DVT
How is HIT diagnosed?
Anti-platelet factor IV or serotonin release assay
What are the clues to diagnosis of BSS?
Adherance by GPIb few large platelets.
What are the clues to diagnosis of glanzman?
Aggregation by GPIIb/IIIa normal platelet count
What are the types of vWF disease?
Type 1 Decreased count Desmopressin Factor 8 concentrate after surgery or trauma
Type 2 Qualitative +/- desmopressin Factor 8 concentrate after surgery or trauma
Type 3 Absent -- Factor 8 concentrate after surgery or trauma
What are the sites of bleeding in hemophilia?
1. Hemarthrosis
2. Intracranial
3. Retroperitoneal
4. Hematuria & hemospermia
5. Intramuscular hematoma
What is the treatment of hemophilia?
Hemophilia A Factor 8 concentrate + desmopressin
Hemophilia B Factor 9 concentrate
What lab tests are used in hemophilia diagnosis?
1. PTT prolonged
2. Factor 8 or 9 level
3. vWF level
4. detection for factor inhibitor mix patient plasma with normal plasma check for PTT correction
What are the normal ranges of coagulation studies?
1. PT 11-15 sec
8. 2. PTT 25-40 sec
3. BT 2-7 min
How to ddx by labs between liver and vit K problems?
1. Liver disease PT and PTT prolonged
2. Vit K def. PT only
Note: normal or elevated fibrinogen R/O DIC
Antithrombin III def. AD Thrombosis
Anti-phospholipid Aquired Arterial & venous thrombosis + thrombocytopenia
Protein C AD Factor 5 & 8
Protein S - Cofactor for protein C
Factor V leiden - --
Prothrombin mutation - --
hyperhomocysteinemia - --
When to suspect inherited hypercoagulable state?
1. Family Hx
2. Recurrent DVT
3. First episode before 40
4. DVT in unusual site (cerebral/mesentric/renal/IVC)
Low grade Small lymphocytic B cell, resembles CLL
Mycosis fungoides T cell, Eczema, cribriform lymphocytes Radio Tx
Follicular small cleaved T(14,18). BCL2, may transform to diffuse large cell Radio Tx
Extranodal MALT
intermediate Follicular large cell --
Diffuse large cell B cell, T cell, mixed CHOP
High grade Lymphoblastic T cell, children, ALL, CNS & skin Chemo Tx
Adult T cell lymphoma HTLV-1, ↑Ca+2
Sezary syndrome T cell, blood (burkit or diffuse-large cell)
Small non-cleaved (Burkit) t(8,14), c-myc, EBV Chemo Tx
immunoblastic T or B cell