Mohammed's Spleen is Palpable

Leukocytosis
1. Causes of Leukocytosis
  1. Nonneoplastic causes
    1. Neutrophilic leukocytosis
      1. Bacterial infections
      2. Corticosteroids
        (inhibit margination)
      3. Myocardial infarction
    2. Eosiniophilia
      1. Type I hypersensitivity reaction
        bronchial asthma
        reaction to penicillin
      2. Helminthic infections
    3. Monocytosis
      1. Chronic infections : Tuberculosis
      2. Autoimmune
        Inflammatory Bowel disease
        Rheumatoid arthritis
        Systemic lupus erythematosus
    4. Lymphocytosis
      1. Viral infections (infectious mononucleosis)
      2. Drugs (phynetoin)
  2. Neoplastic proliferations of the WBCs
    1. Leukemia Vs Lymphoma
      1. Leukemia
        Malignancy of the blood
        Can be in myeloid linage
        Tumor in the blood
        Most common cancer in childhood
        Types
        Acute myeloblastic leukemia(AML)
        Age : 15-60years of age
        2.Thrombocytopenia – bleeding, bruising
        May present as discrete tissue masses (chloroma).
        Myeloblasts contain intracytoplasmic granules (Auer Rods ).
        1.Neutropaenia – fever, chills, infections
        3.Anemia – weakness, fatigue Might have gingival involvement.
        Immunophenotype : CD13+, Myeloperoxidase +
        Acute lymphocytic leukemia(ALL)
        80% of childhood cases (age˂15 years
        composed of immature, precursor B (pre-B) or T (pre-T) lymphocytes referred to as “Lymphoblasts”
        85% of ALLs are precursor B-cell tumours(B-ALL)
        B-ALL: + CD10- CD19- CD20
        Less common, precursor T-cell ALLs(T-ALL) often with thymic involvement.
        T- ALL: + CD2- CD3- CD4-CD8
        Blood film showing blasts
        Abrupt onset –Bone marrow suppression – mass –lymphadenopathy- splenomegaly.
        Symptoms
        Fever-malaise-bleeding (petechial bleeding)-mouth ulcers due to infection.
        Immunophenotyping : + TdT
        Chronic lymphocytic leukemia (CLL)
        60 +-----indolent course(slowly growing tumor)
        Proliferation of neoplastic lymphoid cells (B –cells)
        Widespread involvement of Bm,peripheral blood,LNs,spleen
        hepatosplenomegaly- generalized lymphadenopathy
        Increased WBC count….Absolute lymphocytosis
        Cells are susceptible to destruction …smudge cells in peripheral blood
        Immunedysregulation….Hypogammaglobulinemia
        Transformation to diffuse large B – cell lymphoma
        IHC: CD19+, CD20+, CD23+, CD5+, CD10.
      2. Lymphoma
        Malignancy of the lymphoid tissue
        Only in lymphocytes
        Solid tumor
        Only 10% of childhood cases
        Types
        Hodgkin’s lymphoma
        presence of reed-strenberg cell.
        Non-Hodgkin lymphoma:
        neoplastic proliferation of B or T cells
    2. Chronic Myeloproliferative disorders
      1. Hyperproliferation of neoplastic myeloid progenitors while retaining the capacity for terminal differentiation
        1-Chronic myelocytic leukemia (CML)
        clonal myeloproliferative neoplasm
        Dysregulated production and uncontrolled proliferation of mature and maturing granulocyte with fairly normal differentiation
        Three stages: Chronic Stage Accelerated Stage Blastic Stage
        CML: blood film showing marked leukocytosis , neutrophils at various stages of development (segmented, band , metamyelocytes, myelocytes). The cell in the centre is a basophil (basophilia is prominent in CML) The bone marrow is hypercellular owing to increased numbers of granulocytic and megakaryocytic precursors.
        Fusion of 2 genes
        BCR (or chromosome 22)
        ABL1 (on chromosome 9),
        resulting in BCR-ABL1 fusion gene
        Abnormal chromosome 22 called Philadelphia (Ph) chromosome
        Final product: BCR-ABL1 fusion protein, a dysregulated tyrosine kinase
        Uncontrolled proliferation of transformed cells/ Discordant maturation/ Escape from apoptosis/ Altered interaction with the cellular Matrix
        A subset of patients with CML lack a detectable Ph chromosome but have the fusion product for the bcr/abl translocation detectable by reverse transcriptase- polymerase chain reaction (RT-PCR)
        Management
        Pharmacological
        Tyrosine kinase inhibitors
        Imatinib
        dasatinib
        nilotinib
        bosutinib
        Ponatinib
        Chemotherapy
        Interferon Therapy
        Non pharmacologcai
        Hematopoietic stem cell transplantation
        Radiation Therapy
      2. 2.Polycythemia vera
      3. 3.Myelofibrosis
      4. 4.Essential thrombocythemia
        JAK2 mutation, which normally promote growth and division (JAK/STATA pathway) and it’s important especially for controlling production of blood cells.
2. Leukocytosis refers to an increase in the total number of WBCs due to any cause.
3. if the leukocytosis is so severe, it can mimic leukemia "Leukaemoid Reaction"
  1. How to differentiate between leukemia and a leukmoid reaction
    1. Leukaemoid Reaction
      1. WBC counts up to 15 - 100,000/mm3
        Shift to the left (immature WBCs)
      2. Signs of activation
        Doehle bodies
        toxic granulations
        NAP scores high (Differs the condition from CML)
        MPO negative (Myeloperoxidase) to differ from CML
Hematological History Taking
1. Anemia symptoms
  1. Fatigue, weakness
  2. Blood in stools
  3. Easy bruising
  4. Heavy menstruation
  5. dyspnea
2. Lymphoid symptoms
  1. Infections
3. Past medical, surgical history
  1. Frequent infections
  2. Bleeding disorders
  3. Radiotherapy
  4. Recent trauma causing bleeding
  5. Surgery to GI
  6. Transplant surgery
4. Social history
  1. Smoking
  2. Alcohol
  3. origin
  4. Diet
  5. Sexual lifestyle
5. Drug history
  1. Drug currently taking
  2. OTC’s especially NSAIDs
  3. Anticoagulant therapy
  4. Vitamins, supplements especially Iron, B12
  5. Vitamins, supplements especially Iron, B12
  6. Allergies
  7. Drug allergies
6. Family history
  1. Same condition in a family member
  2. Hemophilia, von willebrand’s
  3. Sickle cell anemia
  4. Thalassemia
  5. Leukemia (Philadelphia chromosome)
Hematological Physical Examination
1. General Appearance
  1. Pale (anemia)
  2. Jaundice (hemolytic anemia).
  3. bruising
2. Hands
  1. pallor of palm
  2. radial pluse
  3. Nails-koilonychia (iron deficiency).
  4. Pale nails (anemia).
3. Forearms
  1. scratch marks
    1. Found on legs as well
  2. purpura (dysglobulinema)
4. Face
  1. Eyes
    1. Conjunctiva: pale (anemia).
    2. Sclera: jaundice (hemolytic anemia)
    3. hemorrhages (polycythemia)
  2. Mouth
    1. Gum hypertrophy (leukemia).
    2. Gum bleeding-scurvy (Vit C).
  3. Tongue:
    1. glossitis (iron deficiency, megaloblastic anemia).
5. Trochlear & axillary nodes
6. Cervical & supraclavicular nodes
7. Rectal and pelvic examination
  1. (blood loss).
8. Fundi
  1. hemorrhage , infection
9. Chest & abdomen
  1. Enlarged liver
10. inguinal nodes
Investigations
1. Blood film
  1. To differ the type of Blast: Myeloblast or Lymphobast (AML or ALL?)
  2. To study the RBC count and morphology
  3. size and shape of the cells in the samples
  4. Determine the Blast count
2. Confirming tests
  1. PCR
    1. is more sensitive than FISH
    2. can be used to monitor the expression quantitively
    3. Polymerase chain reaction is a DNA test that can find the BCR-ABL fusion gene and other molecular abnormalities. PCR tests may also be used to monitor how well treatment is working. This test is quite sensitive and, depending on the technique used, can find 1 abnormal cell mixed in with approximately 1 million healthy cells. This test can be done using a blood sample or bone marrow cells.
  2. FISH
    1. Fluorescence in situ hybridization (FISH) is a test used to detect the BCR-ABL gene and to monitor the disease during treatment. This test does not require dividing cells and can be done using a blood sample or bone marrow cells. This test is a more sensitive way to find CML than the standard cytogenetic tests that identify the Philadelphia chromosome.
  3. Bone marrow aspiration and biopsy
    1. A bone marrow aspiration removes a sample of the fluid with a needle.
    2. A bone marrow biopsy is the removal of a small amount of solid tissue using a needle.
    3. Peripheral blood smear showing leukocytosis with increased number of blasts (MGG-Giemsa stain, x400) (a). Bone marrow aspirate showing megakaryocyte and myeloid cells with excess of blasts (MGG-Giemsa stain, x200, x1000) (b and c). Bone marrow biopsy is hypercellular with myeloid and megakaryocytic hyperplasia (Hematoxylin and Eosin, x400) (d)
3. CBC
  1. To make a total & differential WBC count
    1. Increase in granulocytes
    2. Decrease in Lymphocytes (due to dilution in the differential count) A mild increase in basophils and eosinophils is present
    3. Number of blasts and Promyelocytes and metamyelocytes is ↑
    4. The platelet counts at diagnosis can be low, normal, or even increased in some patients
  2. Determine the Blast count
  3. Too many immature white blood cells ( Total WBC ↑) above 20,000-60,000 cells/μL

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Mohammed's Spleen is Palpable

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	Created by Fatma Shwaylia over 7 years ago