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Hematology: Detailed Information and Notes





Hematology: Detailed Information and Notes


Hematology: Detailed Information and Notes

I. Acute Leukemia (LCMC)

1. Definition:

  • LCMC is a condition characterized by abnormal proliferation of immature cells in the bone marrow, leading to a deficiency in mature cells, affecting blood function.

2. Symptoms:

  • Anemia: Hypochromic anemia, severe, very severe, rapidly progressing.
  • Bleeding: Due to thrombocytopenia, manifested as petechiae, epistaxis, bleeding gums, heavy menstruation, gastrointestinal bleeding…
  • Infection: Due to leukopenia, prone to infections, especially respiratory infections, skin infections, gastrointestinal infections…
  • Ulceration, necrosis: Oral and throat ulcers, tissue necrosis. Creates a distinct foul odor.
  • Infiltration: Enlarged lymph nodes, spleen, liver, skin tumors, gingival hypertrophy.

3. Examination:

  • Bone marrow biopsy: Increased bone marrow proliferation, Blast > 20%, decreased mature bone marrow cells.
  • Cytochemical examination: Using 3 staining methods:
    • PAS: Lymphocytic lineage
    • Peroxidase: Myeloid cells
    • Sudan BLACK: Myeloid cells, stronger than Peroxydase
    • Esterase: Granular lineage cells
  • Genetic testing: Differentiate between monocytic or granular acute myeloid leukemia.
    • t(8,21): M2
    • t(15;17): M3
    • inv(16): M4
    • Ph1 chromosome, polyploidy: Lymphocytic lineage ALL
  • Special examination:
    • DIC: M3
    • Blast < 20%: Detect chromosomal and genetic abnormalities.
* Blast is an immature cell, identified by cytochemical staining methods.
* Bone marrow biopsy is the most important test for diagnosing LCMC, helping to identify the type of abnormal cells, the degree of proliferation, and bone marrow damage.
* Genetic testing helps identify specific chromosomal and genetic abnormalities, helping predict prognosis and select treatment regimens.

4. Treatment:

  • Induction phase:
    • AML: Anthracycline (Daunorubicin) + Cytosine Arabinosid (ARA-C)
    • M3: 3 + 7 regimen plus ATRA (All Trans Retinoic)
  • Consolidation phase: 3+5+7: similar to 3 + 7 plus Epotosid or high-dose cytarabin
  • Maintenance phase:
    • 1/2 induction dose
    • 1 type such as ARA-C or 6MP (common)
  • ALL: Hyper CVAD combined with Course A and Course B, alternating each month for 6-8 months.
    • Course A: Cyclophosphamide, Mesna, Dauno, Vinc, Dexamethasone
    • Course B: Methotrexate, Cytarabine, Calcifolinate
  • CNS infiltration treatment: Spinal tap: MTX + Solu + Cytarabine
  • Side effects:
    • Day 9-14: WBC and platelets drop very low
    • Neutrophil count <50 G/L: Stimulate granulocyte production (G-CSF)
* LCMC treatment regimens are chosen based on the type of LCMC, disease stage, patient’s health status, treatment response, and risk of relapse.
* Patients need to be closely monitored during treatment to detect and manage side effects early.

5. Prognosis:

  • Good prognosis:
    • Children 1-10 years old
    • ALL, WBC not high
    • Chromosomal abnormalities with polyploidy >50 chromosomes
    • Adults:
      • AML with t(8;21) chromosomal abnormality
      • AML/ETO; inv(16); t(15;17)
      • ALL: bone marrow chromosomes > 50
  • Poor prognosis:
    • High WBC
    • Multiple combined chromosomal abnormalities
    • Ph1 chromosome abnormalities
    • FTL3 gene mutation
* Prognosis of LCMC varies widely, depending on many factors such as age, type of disease, disease stage, treatment response, etc.
* Patients need to be followed up regularly to control the disease and detect relapses early.

II. Lymphoma

1. Definition:

  • Lymphoma is a group of cancers that affect the lymphatic system, including lymphocytes (white blood cells).

2. Classification:

  • Hodgkin’s Lymphoma (HK): 60-70% of cases.
  • Non-Hodgkin’s Lymphoma (NKH): 30-40% of cases.

3. Symptoms:

  • Enlarged lymph nodes:
    • HK: Above the diaphragm, in the mediastinum
    • NKH: Both above and below the diaphragm
  • Extra-nodal tumors: D.d, liver, spleen… (common in NKH)
  • Systemic symptoms: Fever, weight loss (over 10% / 6 months), night sweats.

4. Examination:

  • Lymph node imaging: Determine the location and size of lymph nodes.
  • Bone marrow biopsy: Classify cancer cells and assess the degree of invasion.
  • Blood count, bone marrow biopsy, bone marrow biopsy: Determine the degree of bone marrow invasion.
  • Erythrocyte sedimentation rate: Most valuable for predicting prognosis.
  • Uric acid, LDH: Significantly elevated in high-grade lymphoma.
  • Histopathological classification:
    • Lymphocytic predominance: Children, good prognosis
    • Nodular sclerosis (60%): Children, good prognosis
    • Mixed cell type: Average prognosis
    • Lymphocyte depletion: Elderly, worst prognosis
* Bone marrow biopsy is an important test to accurately diagnose the type of lymphoma and assess the degree of invasion.
* High erythrocyte sedimentation rate is a sign of poor prognosis for lymphoma.

5. Treatment:

  • HK:
    • Stage 1 and 2: Good prognosis, chemotherapy, ABVD regimen 4-6 weeks
    • Remaining patients: Chemotherapy alone / Combined chemotherapy and radiotherapy
    • Poor prognosis: MOPP/ABV, ABVD 6 cycles + Radiation
  • NKH:
    • LS stage 1, 2, low grade: Chemotherapy and radiotherapy
    • Elderly patients, average or low risk: COP
    • Average or high grade patients, stage 3, 4: CHOP
  • Targeted therapy: CHOP- Rituximab
  • Relapse / non-responsive: High-dose chemotherapy (DHAP, ICE) combined with autologous stem cell transplantation
* Treatment regimens are chosen based on the type of lymphoma, disease stage, health status, etc.
* Patients need to be followed up regularly to control the disease and detect relapses early.

III. Blood Transfusion

1. Blood Type:

  • Bombay blood type: Lack of H antigen.
  • Rh system: Determined by 3 alleles: Dd, Cc, Ee

2. Use in blood transfusion:

  • HLA platelets: Platelet transfusion, organ transplantation.

3. Blood preservation:

  • Whole blood, red blood cells: 2-6 degrees Celsius.
  • Washed red blood cells: Clean plasma, used for: Autoimmune hemolysis, reaction with plasma proteins.
  • Platelet-rich plasma: 22 degrees Celsius, shake continuously.
  • Fresh frozen plasma (FFP): -25 degrees Celsius, 2 years.

4. Indications for blood transfusion:

  • Whole blood: Blood replenishment, acute blood loss.
  • Red blood cells: Anemia, hemolysis, chronic blood loss.
  • Platelet-rich plasma: Thrombocytopenia, bleeding.
  • FFP:
    • Protein replacement
    • Hemophilia A or B unknown
    • Coagulation disorder
  • Factor VIII precipitate: Hemophilia A, fibrinogen deficiency
  • FFP without precipitate: Hemophilia B, liver failure, burns, protein replacement
* Blood transfusion should be safe and compatible with the patient’s blood type.
* Closely monitor the transfusion reaction after transfusion.

5. Transfusion reactions:

  • Mild: Urticaria, itching, possible chills. Treatment: Stop transfusion, can give antihistamines, continue transfusion after 15-20 minutes.
  • Moderate: Chills, rapid pulse, unchanged or slightly changed BP (<20%). Treatment: Stop transfusion, gradually reduce symptoms: different blood unit, worsen: treat as severe.
  • Severe: Chills, rapid weak pulse, low BP, hemolytic reaction, high fever, delirium, TRALI: 2-6 hours after shortness of breath, pink froth. Treatment: Resuscitation, wrong blood type: add corticosteroids, diuretics, TRALI: increase ventilation, review DIC.
* Transfusion reactions can occur due to many causes: Wrong blood type, immune reaction, infection…
* Timely and accurate management is necessary to prevent dangerous complications.

IV. Idiopathic Thrombocytopenic Purpura (ITP)

1. Definition:

  • Due to the presence of autoantibodies against platelets produced in the spleen.

2. Symptoms:

  • Bleeding in various ages, various forms.

3. Classification:

  • Acute: Often seen in children, after an infection, abrupt onset, 80% spontaneous remission after 2 weeks – 2 months.
  • Chronic: > 6 months, prone to relapses, common in adults.

4. Complications:

  • Retinal hemorrhage: Sign of brain-meningeal hemorrhage.

5. Examination:

  • Bone marrow: Early stage: Increased platelet precursors, late stage: Decreased platelet precursors in the marrow.
  • Coagulation: Prolonged bleeding time, clot does not contract/does not contract completely.
  • Elevated IgG.
  • Platelet lifespan assessment: Radioactive chromium 51.

6. Treatment:

  • Corticosteroids:
    • Usual dose: Pred/Methylpred 1-2 mg/kg/d for 2-4 weeks.
    • Severe, life-threatening: Methylpred 1g/d x 3 days.
  • Immunosuppressants: Azathioprine, Cyclophosphamide, Vincristine (when corticosteroid treatment fails and splenectomy is performed).
  • Splenectomy:
    • Criteria: Corticosteroid treatment failure for 6 months (platelet count < 30), platelet precursor production is still good.
    • Side effects: Increased infection.
    • Prevention: Vaccination before and regularly after surgery: Pneumococcus and H.I.
  • Gamma globulin: Emergency dose: 0.4mg/kg/d x 5 days.
  • Platelet transfusion: Severe bleeding, platelet count < 10 G/L.
  • Plasma exchange: Rapidly reduce anti-platelet antibodies (effective after 2 sessions).
* ITP is a potentially dangerous condition that requires timely treatment and close monitoring.
* High-dose corticosteroids should be used in the acute phase to control the disease.
* Splenectomy is a last resort treatment, only used when medical treatment fails.

V. Hemophilia:

1. Definition:

  • An inherited disease, coagulation disorder due to deficiency of coagulation factor VIII (Hemophilia A) or IX (Hemophilia B).

2. Genetics:

  • Factor 8, 9: Located on the X chromosome.
  • Factor 11: Located on the autosome.

3. Symptoms:

  • Difficult-to-control bleeding, muscle and joint hematomas, mucosal bleeding, recurrent bleeding.
  • Bleeding site: Joint hematomas (knee > elbow > ankle > hip), hematomas in muscles, under the skin, leading to joint deformities due to repeated bleeding.

4. Examination:

  • Prolonged coagulation: Prothrombin time, Howell time, aPTT.
  • Quantification of factor 8, 9: Reduced below 30%.

5. Treatment:

  • Hemophilia B: Transfuse FFP without precipitate / FFP.
  • Hemophilia A: Transfuse concentrated factor 8 / recombinant factor 8 / factor 8 precipitate / FFP.
  • 1 ml of plasma contains: 1 unit of factor 8.
  • Transfuse 1 unit of factor 8 per kg of body weight: Increase factor 8 concentration by 2%.
  • Required factor 8 concentration:
    • Joint and muscle bleeding: 15-20%
    • Trauma: 30-50%
    • Surgery, severe trauma, intracranial bleeding: 80- 100%
  • Drugs that stimulate factor 8 release: Desmopressin 0.3 mg/kg.
  • Factor 8 or 9 treated with heat, chemicals: Inactivate HIV.
* Hemophilia patients need to be followed up regularly to control the disease and detect bleeding early.
* Factor 8 or 9 should be used in the correct dosage and monitored closely after transfusion.

6. Management of bleeding:

  • Compression dressing, use thrombin powder.
  • Clean hematoma, drain hematoma.

7. Genetics:

  • Definitely carrying the Hemophilia gene:
    • Father has it
    • 2 sons have it
    • 1 son has it + 1 male relative has it
  • May carry the Hemophilia gene:
    • Child does not have it but has a maternal blood relationship with the affected person.
    • 1 son has it but no one in the family has it.
* Genetic counseling should be provided for families with a member with Hemophilia.
* Women carrying the Hemophilia gene can give birth to sons with the disease.

VI. Autoimmune Hemolytic Anemia (AIHA):

  • Coombs test positive + elevated LDH: Diagnosis of AIHA.

VII. Red Blood Cell Membrane Disease:

  • Coombs test negative + elevated LDH: Diagnosis of red blood cell membrane disease.

VIII. Paroxysmal Nocturnal Hemoglobinuria (PNH):

  • Ham test positive + elevated LDH: Diagnosis of PNH.

IX. Anemia:

  • Normocytic anemia, normal RBC count: Elevated LDH + Reduced/normal iron: Blood loss.
  • Normocytic anemia, normal RBC count: Elevated iron, elevated bilirubin: Hemolysis.
  • Normocytic anemia, normal RBC count: Elevated LDH + reduced WBC, platelets: Bone marrow failure, hypoplasia.
  • Normocytic anemia, large RBCs: No megaloblasts in the bone marrow + elevated LDH: Hemolysis/acute blood loss.

X. Iron supplements:

  • Siderfol 350 mg 1 tablet/day after meals x 1-2 months.
* Iron supplements should be taken as prescribed by a doctor, avoid self-medication.
* Diagnosis and treatment of hematological conditions require collaboration between a hematologist and the patient.

General note:

  • The information provided in this article is for reference only.
  • Patients should consult a hematologist for accurate diagnosis and treatment.

Hopefully, this article provides comprehensive and detailed information on hematology, helping you better understand related diseases.


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