Diseases Of Blood And Lymphoreticular System Notes and Short Essays

Diseases Of Blood And Lymphoreticular System Important Notes

1. Thalassemia

Diseases Of Blood And Lymphoreticular System Thalassemia

2. Types of leukemia

Diseases Of Blood And Lymphoreticular System Types Of Leukemia

3. Causes of DIC

  • Infections like E.coli, N.meningitis, strep. Pneumonia
  • Cancers of lung, prostrate, pancreas
  • Obstetric preeclampsia, amniotic fluid embolism

4. Complications of polycythemia vera

  1. Elevated red cells
  2. Splenomegaly
  3. Leukocytosis
  4. Thrombosis

5. Components that are destroyed quickly from stored blood are

  • WBC
  • Clotting factors
  • Platelets

6. Types of hemophilia

Diseases Of Blood And Lymphoreticular System Type Of Hemophilia

7. Bleeding tests

Diseases Of Blood And Lymphoreticular System Bleeding Tests

8. Platelets

  • Normal count 1.54 lakhs/mm3
  • Increased in thrombocytosis
  • Decreased in thrombocytopenic purpura

9. Anaemia

Diseases Of Blood And Lymphoreticular System Anaemia

10. Anticoagulants

Diseases Of Blood And Lymphoreticular System Anticoagulants

11. Erythroblastosis foetalis

  • When a Rh ve mother carries in a Rh +ve fetus, usually first child escapes the complication of the Rh incompatibility
  • When the mother conceives for the second time and carries an Rh +ve fetus, the Rh agglutinins enter the fetus and cause agglutination of fetal RBC and hemolysis
  • Complications
    • Hydrops fetalis
    • Kernicterus

Diseases Of Blood And Lymphoreticular System Short Essays

Question 1. Sickle cell anaemia

Answer:

Sickle cell anaemia

  • Hb S is abnormal haemoglobin which gets polymerise and distorts the cell membrane to form abnormal sickle-shaped cell

Sickle cell anaemia Clinical features

  1. Chronic anaemia features
    • Fatigue
    • Lassitude
    • Breathlessness
    • Cardiomegaly
    • Increased susceptibility to infection
    • Hyperplasia of bone marrow producing frontal bossing and malar prominence
    • Mild jaundice
    • Splenomegaly
    • Poor healing ulcers
  2. Other features
    • Pain due to blockage of small or large vessels
    • Large swelling, pain, and tenderness of fingers and toes
    • Acute abdominal colic, renal infarction
    • Painful hematuria
    • Aseptic necrosis of head of femur
    • Fever, malaise
    • Microembolisation of pulmonary vessels occurs in late pregnancy

Read And Learn More: General Medicine Question and Answers

Sickle cell anaemia Diagnosis

  • Haemoglobin low
  • Unconjugated bilirubin increases
  • Peripheral blood smear shows
    • Sickle cell, target cells, and Howell Jowel bodies
  • Positive sickling test
  • Presence of abnormal hemoglobin – HbS

Management

Diseases Of Blood And Lymphoreticular System Sickle Cell Anaemia Management

Question 2. Causes of megaloblastic anaemia

Answer:

Causes of megaloblastic anaemia

  • Inadequate dietary intake
  • Malabsorption
    • Intrinsic factor deficiency
      • Pernicious anaemia
      • Gastrectomy
      • Congenital lack of factor
    • Intestinal causes
      • Tropical sprue
      • Ileal resection
      • Crohn’s disease
    • Removal of B12 from intestine
      • Bacterial proliferation in intestinal blind loop syndrome
      • Fish tapeworm infestations
      • Drugs ex: PAS, neomycin

Question 3. Oral manifestations of leukemia

Answer:

Gingiva

  • Gingivitis
  • Gingival hyperplasia
  • Enlargement of interdental papilla
  • Swollen gingival tissue
  • Cyanotic bluish discoloration of gingiva
  • Thrombosis of gingival vessels

Teeth

  • Rapid loosening of teeth
  • Alteration in developing tooth crypts
  • Destruction of lamina dura
  • Displacement of teeth

Oral mucosa

  • Thinning of oral mucosa
  • Petechiae and ecchymosis over oral mucosa
  • Development of multiple large irregular necrotic ulcers

Other

  • Large hematomas over lower lip
  • Oral infections
  • Palatal ulcerations
  • Mental nerve neuropathy
  • Prolonged postoperative bleeding
  • Osteomyelitis of jaw

Question 4. Iron deficiency anaemia

Answer:

Iron deficiency anaemia

  • It is A chronic, microcytic, hypochromic anacmia that occurs either due to inadequate absorption or excessive loss of iron from the body

Iron Deficiency Anaemia Clinical Features:

  1. Anaemia:
    • Usual symptoms are weakness, fatigue, dyspnoea on exertion, palpitations, pallor of skin, mucous membranes, and sclera.
    • Older patients may develop angina and congestive cardiac failure.
    • Womenmenorrhagia is common symptom.
  2. Epithelial tissue changes:
    • Nails (koilonychia or spoon-shaped nails)
    • Tongue (Atrophic glossitis)
    • Mouth (angular stomatitis)

Iron Deficiency Anaemia  Diagnosis:

  • Serum iron and ferritin are low.
  • Total iron binding capacity is increased Transferrin saturation is below 16%.
  • Stool examination for parasites and occult blood is useful.
  • Endoscopic and radiographic examination of GI tract is needed to detect the source of bleeding.
  • Hematological findings: Examination of peripheral blood picture.
  • Size: Microcytic anisocytotic a Chromicity: Anisochromia is present.
  • Shape: Poikilocytosis often present, pear-shaped tailed variety of RBC, elliptical form common.
  • Reticulocytes: Present, either normal/reduced
  • Osmotic fragility: slightly decreased
  • ESR: Seldom elevated
  • Absolute value: MCV, MCH, and MCEIC are reduced.

Bone marrow findings:

  • Marrow cellularity increased due to erythroid hypoplasia micronormoblast.
  • Marrow ironreduce reticuloendothelial iron stores and absence of siderotic iron granules from developing normoblasts.

Iron Deficiency Anaemia  Management:

  • Iron supplement
  • Ferrous sulphate 300 mg 34 times/day for 6 months only
  • Iron sorbitol 1.5 mg/kg body weight given parentally

Question 5. Aplastic anaemia clinical features

Answer:

Aplastic anaemia

  • Aplastic anemia is characterized by
    • Anaemia
    • Leukopenia
    • Thrombocytopenia
    • Hypocellular bone marrow

Aplastic Anaemia Clinical Features:

  • Anaemia
  • Excessive tendency to bleed
  • Easy bruising
  • Epilaxis
  • Gum bleeding
  • Heavy menstrual flow
  • Petechiae
  • Predisposition to infections

Question 6. Megaloblastic anaemia

Answer:

Megaloblastic anaemia

  • It is macrocytic anaemia with megaloblasts in the bone marrow

Etiology:

  1. Inadequate dietary intake
  2. Malabsorption
    • Intrinsic factor deficiency
      • Pernicious anaemia
      • Gastrectomy
      • Congenital lack of factor
    • Intestinal causes
      • Tropical sprue
      • Ileal resection
      • Crohn’s disease
    • Removal of B12 from the intestines
      • Bacterial proliferation in intestinal blind loop syndrome
      • Fish tape worm infestation
    • Drugs for example, PAS, neomycin

Megaloblastic anaemia Clinical Features:

  • Anemia,
  • Glossitis
  • Neurological manifestations numbness, paraesthesia, weakness, ataxia, diminished reflexes
  • Others mild jaundice, angular stomatitis, purpura, malabsorption, anorexia

Megaloblastic anaemia Management:

  1. Vitamin B12 deficiency
    • Parenteral administration of hydroxocobalamin 1000 microgram twice a week during first week
    • followed by 1000 microgram weekly for 6 weeks
    • Maintenance therapy includes hydroxocobalamin 1000 microgram intramuscular every 3 months for rest of life
  2. folate deficiency
    1. Daily dose of 5 mg of folic acid as initial dose
    2. Dose of 5 mg of folic acid once a week as a maintenance dose

Question 7. Plummer Vinson syndrome

Answer:

Plummer Vinson syndrome

  • It is characterised by dysphagia, iron doccie anaemia, dystrophy of nails and glossits

Plummer Vinson syndrome Clinical Features:

  • Common in middle age females
  • Dysphagia
  • Angular chelitis
  • Spasm in throat
  • Sore mouth
  • A smooth, red, and enlarged tongue
  • Oral mucosa is pale and painful
  • Dry mouth
  • Spoon shaped nails

Plummer Vinson syndrome Diagnosis

  • Biopsy shows:
  • Atrophic epithelium
  • Atrophy of lamina propria and muscles

Plummer Vinson syndrome Management:

  • Correction of anaemia
  • Esophageal dilation

Question 8. Eosinophilia

Answer:

Eosinophilia Clinical features:

  • Dysponea
  • Orthopnoca
  • Wheezing
  • Cough with mucoid expectoration
  • Chest pain

Eosinophilia Management:

  • DiethvlrarbamaZine2 mg/kg three firms a day for 2 weeks
  • Antihistamines are given to treat allergic reations

Question 9. Tropical eosinophilia

Answer:

Tropical eosinophilia

  • It is characterised by the presence of hypereosinophilia, circulating filarial antibodies, microfilaria in tissues
  • Commonly seen in tropical countries like India, and Sri Lanka. Indonesia, Pakistan and Southeast Asia
  • Patient may have lymphadenopathy, splenomegaly,chronic, cough, nocturnal bronchospasm

Tropical eosinophilia Diagnosis:

  • History of prolong stay in endemic area
  • Lack of microfilaria in peripheral blood
  • Peripheral eosinophilia
  • IgE levels of atleast 1000 units/ ml

Tropical eosinophilia Management:

  • Diethylcarbamazine-2 mg/kg three times a day for 2 weeks
  • Antihistamines are given to treat allergic reactions

Question 10. Discuss an approach to bleeding disorders

Answer:

  1. Investigations of disordered vasiular hemostasis
    • Bleeding time
      • It is based on the principle of formation of hemostatic plug following a standard incision on the ulnar aspect of the forearm and the time from incision to when bleeding stops is measured
      • Normal-3-8 minutes
    • Hess capillary resistance test
      • This test is done by tying the sphygmomanometer cuff to the upper arm and raising the pressure in it between diastolic and systolic for 5 minutes
      • After deflation, the number of petechiae appear in the next 5 minutes in 3 camera over the cubital fossa are counted
      • Piesence of more than 20 petechiae is considered a positive test
  2. Investigation of blood coagulation
    • Screening test
      • Whole blood coagulation time
        • The estimation of whole blood coagulation time done by various capillary and tube method
        • Normal value-4-9 minutes
      • Activated partial thromboplastin time (PTTK)
        • This test is used to measure the intrinsic system factors as well as factors common to both intrinsic and extrinsic factors
        • One stage prothrombin time measures the extrinsic system factor 6 as well as factors in the common pathway
    • Special tests
      • Coagulation factor assays
        • These are based on the results of PTTK or prothrombin time tests
      • Quantitative assays
        • Done by immunological and other chemical methods

Question 11. Clinical features and management of acute myeloid leukemia

Answer:

  • Acute myeloid leukemia is a heterogenous disease charaterised by the infiltration of malignant myeloid cells into the blood, bone marrow, and other tissues

Acute Myeloid Leukemia Clinical Features:

  • Anemia-producing pallor, lethargy, dyspnoea
  • Thrombocytopenia
  • Spontaneous bruises
  • Petechiae, bleeding from gums, and other bleeding tendencies
  • Infections
  • Fever
  • Pain and tenderness of bones
  • Lymphadenopathy
  • Splenomegaly
  • Hepatomegaly
  • Leukemic infiltration of the kidney
  • Gum hypertrophy
  • Testicular swelling

Acute Myeloid Leukemia  Management:

  • Regular platelet transfusion
  • Prophylaxis against infections
  • Combination of three drugs is used
    • Cytosine arabinoside
    • Anthracyclines
    • 6-thioguanine
  • Bone marrow transplantation

Question 12. Clinical features and management of chronic myeloid leukemia

Answer:

Chronic Myeloid Leukemia Clinical Features:

  • Onset is usually slow, initial symptoms are often nonspecific.g: weakness, pallor, dyspnoea, and tachycardia.
  • Symptoms due to hypermetabolism such as weight loss, anorexia, night sweats.
  • Splenomegaly is almost always present and is frequently massive. In some patients, it may be associated with acute pain due to splenic infarction.
  • Bleeding tendencies such as bruising, epistaxis, menorrhagia, and heamatomas may occur.
  • Visual disturbance, neurologic manifestations.
  • Juvenile CML is more often associated with lymph node enlargement than splenomegaly.

Chronic Myeloid Leukemia Management:

  • Imatinib oral therapy
  • Allogenic bone marrow transplantation
  • Interferon-alpha
  • Chemotherapy drugs used are busulfan, cyclophosphamide, and hydroxyurea

Question 13. Oral manifestations of hematological disorders transfusion

Answer:

Oral manifestations of hematological disorders transfusion

  • Gingivasevere hemorrhage
  • Soft tissue hematoma formation
  • Jawrecurrent subperiosteal hematoma
  • Tumourlike malformation B Teethhigh caries index
  • Severe periodontal disease
  • Oropharyngeal bleeding
  • Severe bleeding at the injection site

Question 14. Complications of blood transfusion

Answer:

Complications of Blood Transfusion

  1. Transfusion reactions
    • Incompatibility due to
      • Incompatible transfusion
      • Transfusion of hemolysed blood
      • Transfusion of expired blood
    • Pyrexia reactions characterized by
      • Pyrexia
      • Chill
      • Rigor
      • Restlessness
      • Headache
      • Increased pulse rate
      • Nausea and vomiting
    • Allergic reactions
      • Mild tachycardia
      • Urticarial rash
      • Fever
      • Dyspnoea
    • Sensitization to leucocytes and platelets
  2. Transmission of diseases like
    • Hepatitis
    • AIDS
    • Bacterial infections
  3. Reactions caused by massive transfusion
    • Acid base imbalance
    • Hyperkalaemia
    • Citrate toxicity
    • Hypothermia
    • Failure of coagulation
  4. Complication of general intravenous fluid transfusion
    • Congestive cardiac failure
  5. Complication of general intravenous fluid transfusion
    • Thrombophlebitis
    • Air embolism

Question 15. Idiopathic thrombocytopenic purpura investigations and treatment

Answer:

. Idiopathic thrombocytopenic purpura

  • Idiopathic thrombocytopenic purpura is an autoimmune disorder due to antibodies directed against platelet membrane glycoprotein resulting in the premature clearance of these cells by the immune system

Idiopathic Thrombocytopenic Purpura  Investigations:

  • Chronic history of bleeding
  • Bleeding time is prolonged
  • Reduced platelet count
  • Bone marrow shows increased megakaryocytes indicating peripheral destruction of platelets

Idiopathic Thrombocytopenic Purpura  Management:

  • Self-limiting in children
  • Moderate to severe ITP is treated with prednisolone 2 mg/ kg daily orally
  • In adults prednisolone 60 mg dialysis started and then continued for 24 weeks and then slowly withdrawn
  • In severe cases, intravenous immunoglobulin IgG should be given in a dose of 1 g/kg

Diseases Of Blood And Lymphoreticular System Short Answers

Question 1. Sickle cell anaemia

Answer:

Sickle cell anaemia

  • Hb S is abnormal haemoglobin which gets polymerise and distort the cell membrane to form abnormal sickle-shaped cell

Sickle cell anaemia Clinical features

  • Chronic anaemia features
    • Fatigue
    • Lassitude
    • Breathlessness
    • Cardiomegaly
    • Increased susceptibility to infection
    • Hyperplasia of bone marrow producing frontal bossing and malar prominence
    • Mild jaundice
    • Spleenomegaly
    • Poor healing ulcers
  • Other features
    • Pain due to blockage of small or large vessels
    • Large swelling, pain and tenderness of fingers and toes
    • Acute abdominal colic, renal infarction
    • Painful hematuria
    • Aseptic necrosis of head of femur
    • Fever, malaise
    • Microembolisation of pulmonary vessels occurs in late pregnancy

Question 2. Von Willebrand disease

Answer:

Von Willebrand disease

  • It is coagulation disorder

Von Willebrand disease Causes and related features

  • Platelet dysfunction leads to
    • Superficial bruising
    • Epitaxis
    • Menorrhagia
    • GI bleeding
  • Low levels of von Willebrand factor
    • Depending on the level of vWF, 3 types of disease occurs

Von Willebrand disease Diagnosis:

  • Abnormal platelet function
  • Increased bleeding time
  • Normal prothrombin time
  • Decreased factor 8
  • Reduced vWF

Question 3. Causes of pancytopenia

Answer:

Pancytopenia

  • Pancytopenia is a simultaneous presence of anaemia, leucopenia, and thrombocytopenia
  • pancytopenia Causes
    • Aplastic anaemia
    • Paroxysmal nocturnal hemoglobinuria
    • Megaloblastic anaemia
    • Bone marrow infiltration

Question 4. Peripheral smear in iron deficiency anaemia

Answer:

Peripheral smear in iron deficiency anaemia

  • Hematological findings: Examination of peripheral blood picture.
  • Size: Microcytic anisocytosis
  • Chromicity: Anisochromia is present.
  • Shape: Poikilocytosis is often present, pear-shaped tailed variety of RBC, elliptical form common.
  • Reticulocytes: Present, either normal/reduced
  • Osmotic fragility: slightly decreased
  • ESR: Seldom elevated
  • Absolute value: MCV, MCH, and MCHC are reduced.

Question 5. Treatment of iron deficiency anaemia

Answer:

Iron supplement

  • Ferrous sulphate 300 mg 34 times/day for 6 months only
  • Iron sorbitol 1.5 mg/kg body weight given parentally

Question 6. Iron therapy

Answer:

Iron Preparations:

  1. Oral iron preparations
    • It is the preferred route of administration
    • Preparations:
      • Ferrous sulfate 200 mg tab
        • Contains 20% hydrated salt and 32% dried salt
        • It is inexpensive
        • Produces metallic taste in mouth
      • Ferrous gluconate 300 mg tab
        • Contains 12% of iron
        • Causes less gastric irritation
      • Ferrous fumarate 200 mg tab
        • Contains 33% iron
        • It is less water soluble and tasteless
      • Ferrous succinate 100 mg tab, 35% iron
        • Better absorbed and expensive
      • Iron calcium complex 5% iron
        • Better absorbed and expensive
      • Ferric ammonium citrate 45 mg tab
        • Better absorbed and expensive
    • Dose:
      • Total 200 mg given daily in 3 divided doses
      • Prophylactic dose 30 mg daily
    • Indications:
      • Iron deficiency anaemia
    • Adverse Effects:
      • Nausea, vomiting, epigastric pain
      • Heart bum
      • Staining of teeth
      • Metallic taste
      • Constipation
  2. Parenteral iron preparations
    • Intramuscular injection of iron is given deep in the gluteal region using Z technique
    • Intravenous is given slowly over 510 minutes
    • Preparations:
      • Iron dextran
        • It is colloidal solution containing 50 mg elemental iron
        • It is given intravenously and Intramuscularly
      • Iron sorbitol citric acid complex
        • Contains 50 mg elemental iron
        • Can be given only IM
        • If given 4 it quickly saturates transferrin stores
        • Due to it free iron levels in the plasma rises and causes toxicity
    • Dose:
      • It is calculated using formula:
      • Iron requirement ( in mg) = 4.4 * body weight (in kg) * Hb deficit (in g/ dl)
    • Indications:
      • When oral iron is not tolerated
      • Failure of absorption of oral iron
      • Noncompliance
      • In presence of severe deficiency with chronic bleeding
      • Along with erythropoietin
    • Adverse Effects:
      • Pain at the site of injection
      • Pigmentation of skin
      • Sterile abscess
      • Fever
      • Headache
      • Joint pain, flushing, palpitation
      • Chest pain, dyspnoea
      • Anaphylactic reaction

Question 7. Megaloblastic anaemia

Answer:

Megaloblastic anaemia

  • It is macrocytic anaemia with megaloblastosis in the bone marrow

Etiology:

  1. Inadequate dietary intake
  2. Malabsorption
    • Intrinsic factor deficiency
      • Pernicious anaemia
      • Gastrectomy
      • Congenital lack of factor
    • Intestinal causes
      • Tropical sprue
      • Ileal resection
      • Crohn’s disease
    • Removal of B12 from the intestines
      • Bacterial proliferation in intestinal blind loop syndrome
      • Fish tape worm infestation
    • Drugs, for example, PAS, neomycin

Megaloblastic anaemia Clinical Features:

  • Aneamia
  • Glossitis
  • Neurological manifestations numbness, paraesthesia, weakness, ataxia, diminished reflexes.
  • Othersmild jaundice, angular stomatitis, purpura, malabsorption, anorexia

Question 8. Four causes of anaemia

Answer:

Four causes of anaemia

  1. Anaemia due to increased blood loss
    • Acute posthaemorrhagic anaemia
    • Chronic blood loss
  2. Anaemias due to impaired red cell production
    • Cytoplasmic maturation defects
      • Deficient haem synthesis:
        • Iron deficiency anaemia
      • Deficient globin synthesis:
        • Thalassaemic syndromes
    • Nuclear maturation defects
      • Vitamin B12 and/or folic acid deficiency:
      • Megaloblastic anaemia
    • Defect in stem cell proliferation and differentiation
      • Aplastic anaemia
      • Pure red cell aplasia
    • Anaemia of chronic disorders
    • Bone marrow infiltration
    • Congenital anaemia
  3. Anaemias due to increased red cell destruction (Haemolytic anaemias).
    • Extrinsic (Extracorpuscular) red cell abnormalities
    • Intrinsic (Intracorpuscular) red cell abnormalities

Question 9. Causes of iron deficiency anaemia

Answer:

Causes of iron deficiency anaemia

  • Inadequate intake of iron in diet
  • Malabsorption of iron due to diarrhea
  • Increased requirement in growing child and in pregnanacy
  • Increased loss of iron due to injury, epitaxis, and peptic ulcer
  • Gastrotomy

Question 10. Hemolytic anaemia

Answer:

Hemolytic anaemia Definition:

  • It is an abnormal exessive destruction of red cells that overwhelms the compensatory capacity of the bone marrow

Etiology:

  1. Congenital
    • Abnormalities of red cell membrane
    • Disorders of haemoglobin
    • Red cell enzyme defects
  2. Acquired
    • Immune defects
    • Nonimmune defects

Question 11. Two drugs used in eosinophilia

Answer:

Two drugs used in eosinophilia

  • Drugs used in eosinophilia are;
  1. Prednisolone
  2. Diethylcarbamazine

Question 12. Classification of leukemia

Answer:

leukemia

  1. Based on cell types predominantly involved.
    • Myeloid
    • Lymphoid.
  2. Based on natural history of disease:
    • Acute
    • Chronic.

WHO classification of myeloid neoplasm:

  1. Myeloproliferative Diseases:
    • Chronic myeloid leukaemia (CML), {Ph chromosome t(9;22), BCR/ABLpositive}
    • Chronic neutrophilic leukaemia
    • Chronic eosinophilic leukaemia/ hypereosinophilic syndrome
    • Chronic idiopathic myelofibrosis
    • Polycythaemia vera (PV)
    • Essential thrombocythaemia (ET)
    • Chronic myeloproliferative disease, unclassifiable
  2. Myelodysplastic/Myeloproliferative Diseases:
    • Chronic myelomonocytic leukaemia (CMML)
  3. Myelodysplastic Syndrome (MDS):
    • Refractory anaemia (RA)
    • Refractory anaemia with ring sideroblasts (RARS)
    • Refractory cytopenia with multilineage dysplasia (RCMD)
    • RCMD with ringed sideroblasts (RCMD-RS)
    • Refractory anaemia with excess blasts (RAEB-1)
    • RAEB-2
    • Myelodysplastic syndrome unclassified (MDS-U)
    • MDS with isolated del 5q
  4. Acute Myeloid Leukaemia (AML):
    • AML with recurrent cytogenetic abnormalities
      • AML with t(8;21)
      • AML with abnormal bone marrow eosinophils
      • Acute promyelocytic leukaemia (t(15;17)
      • AML with 11q23 abnormalities (MLL)
    • AML with multilineage dysplasia
      • With prior MDS
      • Without prior MDS
    • AML and MDS, therapy-related
      • Alkylating agent related
      • Topoisomerase type 2 inhibitor-related
      • Other types
    • AML, not otherwise categorised
      • AML, minimally differentiated
      • AML without maturation
      • AML with maturation
      • Acute myelomonocytic leukaemia (AMML)
      • Acute monoblastic and monocytic leukaemia
      • Acute erythroid leukaemia
      • Acute megakaryocytic leukaemia
      • Acute basophilic leukaemia
      • Acute panmyelosis with myelofibrosis
      • Myeloid sarcoma
  5. Acute Biphenotypic Leukaemia

Question 13.Chronic myeloid leukemia

Answer:

Chronic myeloid leukemia Clinical Features:

  • Onset is usually slow, initial symptoms are often nonspecific.g: weakness, pallor, dyspnoea, and tachycardia.
  • Symptoms due to hypermetabolism such as weight loss, anorexia, and night sweats.
  • Splenomegaly is almost always present and is frequently massive. In some patients, it may be associated with acute pain due to splenic infarction.
  • Bleeding tendencies such as bruising, epistaxis, menorrhagia, and heamalomas may occur.
  • Visual disturbance, neurologic manifestations.
  • Juvenile CML is more often associated with lymph node enlargement than splenomegaly.

Question 14. Agranulocytosis

Answer:

Agranulocytosis

  • The term agranulocytosis is used to describe a stale of severe neutropenia.
  • A reduction in the number of granulocytes in blood is known as neutropenia.

Agranulocytosis Causes:

  • Neutropenia can be caused by following reasons.
    1. Drug induced: Anti-cancer, antibiotics, anticonvulsants, antithyroid drugs
    2. Haematological disease: Aplastic anaemia, acute leukaemia etc.
    3. Infections: Malaria, TB, typhoid,
    4. Autoimmune: Systemic lupus erythematosis
    5. Congenital: Cyclic neutropenia.

Agranulocytosis Clinical Features:

  • Initally patient develops malaise, chills, and fever, with subsequently marked weakness and fatiguability.
  • Massive growth of microorganisms due to inability to produce leukocyte response.
  • Infections are usually present as ulcerating, necrotizing lesions of gingiva, the floor of the mouth, buccal mucosa, and other sites within oral cavity known as agranulocytic angina.
  • Lymphadenopathy and hepatosplenomegaly may be present.

Agranulocytosis Treatment:

  • Patients with neutropenia and fever must be sent to the hospital.
  • In severe infections, neutrophil transfusion must be done.
  • Broad-spectrum antibiotics and antifungal drugs are to be given.

Question 15. Management of acute leukemia

Answer:

Management of acute leukemia

  • Regular platelet transfusion
  • Prophylaxis against infections
  • Combination of three drugs is used
    • Cytosine arabinosidc
    • Anthracyclines
    • 6thioguanine
  • Bone marrow transplantation

Question 16. Purpura

Answer:

Purpura Types:

  1. Nonthrombocytopenic purpura
    • It is due to defect of capillaries
  2. Thrombocytopenic purpura
    • It is due to decreased number of platelets

Purpura Clinical Features:

  • Antiplatelet globulin or antibodies to platelets in blood are present
  • Spleen is not palpable
  • Severe and profuse gingival bleeding
  • Platelet count is less than 60,000 per mm2
  • Increased bleeding time
  • Cessation of bleeding

Purpura Treatment:

  • Splenectomy

Question 17. Thrombotic thrombocytopenic purpura

Answer:

Thrombotic thrombocytopenic purpura

  • It is lethal disorder of vascular endothelial damage

Thrombotic thrombocytopenic purpura Cause:

  • Deficiency of a protease called ADAMTS 13

Thrombotic thrombocytopenic purpura Clinical Features

  • Florid purpura
  • Fibrin mierolhrombi
  • Thrombocytopenia
  • Macroangiopalhic hemolytic anaemia
  • Fever
  • Renal failure
  • Fluctuating levels of consciousness
  • Focal neurological signs

Thrombotic thrombocytopenic purpura Treatment:

  • Exchange transfusion
  • Plasmapheresis combined with infusion of fresh frozen plasma

Question 18. Idiopathic thrombocytopenic purpura

Answer:

Idiopathic thrombocytopenic purpura

  • It is characterized by Immunologic destruction of platelet and normal or increased megakaryocytes in the bone marrow

Idiopathic thrombocytopenic purpura Types:

  • Acute ITP
  • Chronic ITP

Idiopathic thrombocytopenic purpura Clinical Features:

  • Usual manifestations are petechial haemorrhage, purpura, easy bruising, epistaxis, mucosal bleeding such as menorrhagia in women, nasal bleeding, bleeding from gums, haematuria.
  • Intracranial haemorrhage is rare.
  • Splenomegaly, hepatomegaly may also occur.

Question 19. Importance of hemophilia to dental surgeon

Answer:

Importance of hemophilia to dental surgeon

  • It is essential to prevent accidental damage to the oral mucosa when carrying out any procedure in the mouth
  • Blood loss can be controlled locally with direct pressure or periodontal dressings with or without topical anlifibrinolytie agents
  • Patients with bleeding disorders can be given dentures as long as they are comfortable
  • Fixed and removable orthodontic appliances may be used along with regular preventive advice and hygiene therapy
  • Endodontic treatment is generally low risk for patients with bleeding disorders
  • Aspirin should not be used

Question 20.Burkitt’s lymphoma

Answer:

Burkitt’s lymphoma

  • Burkitt’s lymphoma is a rare type of nonHodgkin’s lymphoma (NHL).
  • Epsteinbarr virus (EBV) and HIV are associated with the development of Burkitt’s lymphoma.
  • This is the most rapidly progressive tumour first described in African children.
  • Most patients present with lymphadenopathy and abdominal mass.
  • It has a tendency to metastasize to the CNS.
  • Treatment should be initiated promptly with intensive chemotherapy. Prophylactic chemotheiapy is also given. 70-80% patients may be cured.

Question 21. Drugs used in Hodgkin’s lymphoma

Answer:

Drugs used in Hodgkin’s lymphoma are

  1. Vinblastin sulfate
  2. Bleomycin
  3. Mustargen
  4. Chlorambucil
  5. Cyclophosphamide

Question 22. Hyperspleenism

Answer:

Hyperspleenism

  • It is defined as a clinical condition in which spleen removes excessive quantites of erythrocytes, granulocytes, and platelets from the circulation

Hyperspleenism Clinical Features:

  • Cirrhosis of liver with portal hypertension
  • Accelerated destruction of one or more elements of blood
  • Pancytopenia
  • Infections
  • Easy bruising
  • Excessive bleeding tendencies

Hyperspleenism Treatment:

  • Treatment of underlying disorder
  • Spleenectomy
  • Supportive antibiotics
  • Blood transfusion

Question 23. Spleenomegaly

Answer:

Spleenomegaly

  • Enlargement of spleen is called spleenomegaly

Spleenomegaly Causes:

  1. Infective disorders
    • Bacterial
      • Endocarditis
      • Tuberculosis
      • Septicaemia
      • Salmonella
    • Viral
      • Hepatitis
      • AIDS
      • Infectious mononucleosis
    • Protozoal
      • Malaria
      • Leishmaniasis
    • Spirochaetal
      • Syphilis
    • Fungal
      • Histoplasmosis
  2. Inflammatory disorders
    • Rheumatoid arthritis
    • SLE
    • Sarcoidosis
  3. Congestive splenomegaly
    • Hepatic vein thrombosis
    • Chronic congestive heart failure
    • Pericardial effusion
  4. Diseases associated with hemolysis
    • Spherocytosis
    • Sickle cell disease
  5. Infiltrative disease of spleen
  6. Miscellaneous
    • Iron deficiency anaemia
    • Idiopathic
    • Sarcoidosis

Question 24. Causes of massive spleenomegaly

Answer:

Causes of massive spleenomegaly

  • Hemolytic anaemia thalassaemia
  • Infectionsmalaria, kalaazar
  • Collagen vascular disorder rheumatoid arthritis
  • Congestionportal hypertension
  • Malignant infiltration leukemia

Question 25. Bone marrow aspiration

Answer:

Bone marrow aspiration

  • Bone marrow aspiration is used for bone marrow examination

Bone marrow aspiration Uses:

  • To study cell markers
  • Karyotyping
  • Accurate diagnosis
  • Assessment of malignant diseases
  • Assessment of cellularity
  • Details of developing blood cells
  • Detect ratio between erythroid and myeloid cells
  • Assess iron stores

Bone marrow aspiration Indications:

  • Infiltrative disorders
  • Parasitic diseases
  • Cytopenic disorders
  • Infective disorders

Question 26. ESR

Answer:

ESR

If a sample of blood with an anticoagulant is allowed to stand in a vertical tube, the RBC settles down due to gravity as compared to plasma with a clear supernatant layer of plasma. The rate at which the cells settle down is called ESR.

Method of Estimation of ESR:

There are two standard methods as follows:

  1. Wintrobe’s and Landsberg’s method: ESR is measured in undiluted blood in a hematocrit tube.
  2. Westergren’s method: ESR is measured in venous blood diluted accurately with 31.3 g/L trisodium citratein the proportion of 1:4 (1 volume of 3.8% sodium citrate and 4 volume of blood).

The conditions were ESR is markedly raised are as follows:

  1. Infective: Tuberculosis, kala-azar, in most of the chronic infections.
  2. Inflammation: Rheumatoid arthritis, rheumatic fever, other connective tissue disorders.
  3. Neoplastic: Multiple myeloma, lymphoma, paraproteinaemia.
  4. Miscellaneous: Aplastic anaemia, autoimmune disorders, mixed connective tissue disorders.

Question 27. Three causes of neutropenia

Answer:

Three causes of neutropenia

Neutropenia can be caused by following reasons.

  1. Drug-induced: Anti-cancer, antibiotics, anticonvulsants, antithyroid drugs
  2. Haematological disease: Aplastic anaemia, acute leukaemia etc.
  3. Infections: Malaria, TB, typhoid.
  4. Autoimmune: Systemic lupus erythematosis
  5. Congenital: Cyclic neutropenia.

Question 28. Folic acid deficiency

Answer:

Folic acid deficiency

  • Folic acid deficiency leads to:
  • Megaloblastic anaemia
  • Glossitis
  • Diarrhoea
  • Weakness

Question 29. Blood groups

Answer:

Blood groups Classification:

  • It is based on the presence or absence of a specific agglutinogen or antigen on the surface of the red cell membrane
  • Important blood groups are:
  1. ABO system
    • It is based on the presence or absence of antigen A and antigen B
  2. Rh blood group
    • Rh fator is an antigen present in RBC

Blood groups Uses:

  • In blood transfusion
  • For matching tissues in organ transplantation
  • Investing cases of paternity disputes
  • Helpful in emergency conditions
  • In pregnancy
  • For research purposes
  • Anthropological purposes

Question 30. Hematological and bone marrow findings of multiple myeloma

Answer:

Hematological and bone marrow findings of multiple myeloma

  1. Blood examination shows
    • Anaemia
    • Neutropenia
    • Thrombocytopenia
    • Raised ESR
    • Reversal of albumin globulin ratio
    • Presence of BenceJones proteins
  2. Bone marrow examination
    • Exhibits presence of at least 10% atypical plasma cells in the total marrow cell population

Question 31. Clotting time

Answer:

Clotting time

  • It is the time interval from oozing of blood after a cut or injury till arrest of bleeding

Normal Duration:

  • 3-8 minutes

Prolonged In:

  • Hemophilia

Question 32. Prothrombin time

Answer:

Prothrombin time

Prothrombin time measures the extrinsic system factor 7 as well as factors in the common pathway

  • In it, tissue thromboplastin and calcium are added to the test

Normal Time:

  • 10-14 seconds

Prolonged in:

  • Administration of oral Anticoagulants
  • Liver disease
  • Vitamin K deficiency
  • Disseminated intravascular coagulation

Question 33. DIC

Answer:

DIC

Disseminated intravascular coagulation is a complex thrombo-hemorrhagic disorder occurring as a secondary complication in some systemic diseases

Etiopathogenesis:

  • Pathogenesis of DIC includes following events
  1. Activation of coagulation
    • Etiological factors like massive tissue injury, presence of infections, endothelial damage causes activation of coagulation by release of tissue factor
  2. Thrombotic phase
    • Endothelial damage from various thrombogenic stimuli causes:
      • Generalized platelet aggregation
      • Platelet adhesion
      • Deposition of small thrombi and emboli throughout microvasculature
  3. Consumption phase
    • Consumption of coagulation factors and platelets occurs
  4. Secondary fibrinolysis
    • Fibrinolytic system is secondary activated
    • This causes the breakdown of fibrin resulting in formation of FDPs in circulation

Question 34. Folic acid

Answer:

Folic acid

Folic acid is important in one-carbon metabolism

Folic acid Sources:

  • Green vegetables, liver, yeast, egg, milk, and some fruits

Folic acid Functions:

  • The Coenzyme of it, tetrahydrofolate (THF), serves as an acceptor or donor of one carbon units
  • It is involved in the synthesis of important compounds like
  • PurinesIncorporated into DNA and RNA
  • Pyrimidine nucleotide-deoxy thymidylic acid involved in the synthesis of DNA
  • Aminoacids-Glycine, serine, ethaloamine, and choline
  • N-FormylmethionineInitiator of protein biosynthesis

Folic acid Deficiency Manifestations:

  • Megaloblastic anaemia
  • Glossitis
  • Diarrhoea
  • Weakness

VIVA VOCE

  1. Chlorambucil is drug of choice of chronic lym, phoid leukemia
  2. Busulfan, hydroxycarbamide are drug of choice in chronic myeloid leukemia
  3. Hemophilia is transmitted as X-linked recessive trait
  4. Von Willebrand’s disease is transmitted as autosomal dominant trait

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