Diseases Of Blood And Lymphoreticular System Long Essays

Diseases Of Blood And Lymphoreticular System Long Essays

Question 1. Classify anemia and its diagnostic approach. 

Answer:

Anaemia  Classification

1. Pathophysiologic:
   • Anaemia due to increased blood loss
     • Acute posthaemorrhagic anaemia
     • Chronic blood loss
   • Anemias due to impaired red cell production
     • Cytoplasmic maturation defects
       • Deficient haem synthesis: Iron deficiency anemia
       • Deficient globin synthesis: Thalassaemic syndromes
     • Nuclear maturation defects
       • Vitamin B12 and/or folic acid deficiency: Megaloblastic anemia
     • Defects in stem cell proliferation and differentiation
       • Aplastic anemia
       • Pure red cell aplasia
     • Anemia of chronic disorders
     • Bone marrow infiltration
     • Congenital anaemia
   • Anemias due to increased red cell destruction (Haemolytic anemias).
     • Extrinsic (Extracorpuscular) red cell abnormalities
     • Intrinsic (Intracorpuscular) red cell abnormalities
2. Morphologic:
   • Microcytic, hypochromic
   • Normocytic, normochromic
   • Macrocytic, normochromic
3. Diagnosis:
   • Laboratory diagnosis of anemia includes
     • Peripheral blood smear
       • It shows
         • Variations in the size of RBCs-microcytic or macrocytic
         • Variations in the shape of RBCs poikilocytosis
         • Spherocytosisspindle shaped RBCs
         • Nucleated RBCs
         • Inadequate hemoglobin formation
         • Presence of HowellJolly bodies
         • Irregularly contracted red cells
   • Hemoglobin content
     • Hemoglobin content decreases in anemia
   • Red cell indices are used like MCV, MCH, MCHC
   • ESR estimation
   • Bone marrow aspiration
   • Leucocyte and platelet count
   • Reticulocyte count

Question 2. Describe the etiological factors, clinical features, and management of iron deficiency anemia,
(or)
Outline the causes of iron deficiency anemia and how to manage such a case
(or)
Classify anemia. Describe clinical features, diagnosis, and management of iron deficiency anemia
(or)
Enumerate causes of iron deficiency anemia. Describe its clinical features and findings in the peripheral blood smear of this condition and treatment

Answer:

Iron Deficiency Anaemia:

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

Iron Deficiency Anaemia Causes:

• Inadequate intake of iron in the diet
• Malabsorption of iron due to diarrhea
• Increased requirements in a growing child and pregnancy
• Increased loss of iron due to injury, epistaxis, and peptic ulcer
• Gastrotomy

Iron Deficiency Anaemia Clinical Features:

Iron Deficiency Anaemia Diagnosis:

• Serum iron and ferritin are low.
• Total iron binding capacity is increased n Transferrin saturation is below 16%.
• Stool examination for parasites and occult blood is useful.
• Endoscopic and radiographic examination of the GI tract is needed to detect the source of bleeding.
• Hematological findings: Examination of peripheral blood picture.
• Size: Microcytic anisocytosis
• Chromicity: Anisochromia is present.
• Shape: Poikilocytosis is often present, a 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.

Read And Learn More: General Medicine Question and Answers

Bone Marrow Findings:

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

Bone Marrow Findings Management:

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

Question 3. Describe etiological factors, clinical features, and management of megaloblastic anemia

Answer:

Megaloblastic Anaemia:

• It is macrocytic anemia with megaloblasts in the bone marrow

Etiology:

1. Inadequate dietary intake
2. Malabsorption
   • Intrinsic factor deficiency
     • Pernicious anemia
     • 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 tapeworm infestation
   • Drugs, for example, PAS, neomycin

Megaloblastic Anaemia Clinical Features:

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

Megaloblastic Anaemia Diagnosis:

1. Blood picture:
   • Hemoglobin concentration falls.
   • MCV and MCH increases
   • MCHC decreases/remains normal.
   • Reticulocyte count is low
   • Red blood cell blood smear demonstrates anisocytosis, poikilocytes, and the presence of macroovalocytes
   • Leucocytes’ total WBC count is less
   • Thrombocytes giant platelets are present.
2. Bone marrow findings:
   • Marrow cellularity hypercellular bone marrow with decreased myeloid: erythroid ratio.
   • Erythropoiesis erythroid hyperplasia is due to characteristic megaloblastic erythropoiesis.
   • Megaloblasts are abnormal, large, nucleated erythroid precursors, having nuclear-cytoplasmic asynchrony. Nuclei are large, having fine reticular and open chromatin,
   • Abnormal mitosis may be seen in megaloblasts.
   • Marrow iron increases in the number and size of the iron granules in erythroid precursors. Iron in reticulum cells is increased.

Megaloblastic Anaemia Management:

1. Vitamin B12 deficiency
   • Parenteral administration of hydroxocobalamin 1000 microgram twice a week during the first week
   • Followed by 1000 micrograms weekly for 6 weeks
   • Maintenance therapy includes hydroxocobalamin 1000 microgram intramuscular every 3 months for the rest of life
2. Folate deficiency
   • A daily dose of 5 mg of folic acid as an initial dose
   • A dose of 5 mg of folic acid once a week as a maintenance dose

Question 4. Describe the differential diagnosis of megaloblastic anemia. Add a note on the treatment of pernicious anemia

Answer:

Differential Diagnosis of Megaloblastic Anaemia:

1. Iron deficiency anemia
   • Clinical Features:
     • 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 a common symptom.
     • Epithelial tissue changes:
       • Nails (koilonychia or spoon-shaped nails)
       • Tongue (Atrophic glossitis)
       • Mouth (angular stomatitis)
2. Thrombocytopenia
3. Gastritis
4. Peripheral neuropathy
   • Numbness, paraesthesia, weakness, ataxia, diminished reflexes.

Treatment of Pernicious Anaemia:

• Parenteral administration of vitamin B12
• Physiotherapy for neurologic deficits B Blood transfusion
• Follow-up visits for early detection of cancer of the stomach
• Corticosteroid therapy to improve gastric lesions

Question 5. Mention causes of aplastic anemia. Describe its clinical features, diagnosis, complications, and management.

Answer:

Aplastic anemia

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

Etiology:

• Idiopathic
• Secondary to drugs, viruses, pregnancy
• Hereditary

Aplastic Anaemia Clinical Features:

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

Aplastic Anaemia  Investigations:

• Blood smear shows normocytic, microcytic anemia, decreased granulocytes, and platelet count
• Chromosomal studies for inherited disorders

Aplastic Anaemia  Complications:

• Bleeding
• Infection
• Death within 6-12 months

Aplastic Anaemia  Treatment:

• Bone marrow transplantation
• Immunosuppressive therapy
• Packed red cell transfusions
• Granulocytes transfusions

Question 6. Describe etiological factors, clinical features, and management of polycythemia vera

Answer:

Polycythaemia Vera:

• It is a clonal disorder characterized by increased production of all myeloid elements resulting in pinocytosis

Etiology:

• Chromosomal abnormalities like 20q, trisomy 8 and 9p

Polycythaemia Vera Clinical Features:

• Headache
• Vertigo
• Tinnitus
• Visual disturbances
• Increased risk of thrombosis
• Increased risk of hemorrhages
• Splenomegaly
• Pruritis
• Increased risk of urate stones and gout

Polycythaemia Vera Management:

• Phlebotomyto reduce total blood cell count
• Anticoagulant therapy to treat thrombosis
• Chemotherapy to induce myelosuppression
• Use of uricosuric drugs to treat hyperuricemia
• Interferon-alpha

Question 7. Classify leukemias. Describe the clinical features and management of one of them
(or)
Classify leukemias. Outline clinical features and diagnosis of chronic myeloid leukemia
(or)
Describe etiology, clinical features, and management of chronic myeloid leukemia

Answer:

Leukaemias Classification:

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

WHO classification of myeloid neoplasm:

1. Myeloproliferative Diseases:
   • Chronic myeloid leukaemia (CML), {Ph chromosome t(9;22) (q34;2), BCR/ABLpositive}
   • Chronic neutrophilic leukemia
   • Chronic eosinophilic leukemia/ hypereosinophilic syndrome
   • Chronic idiopathic myelofibrosis
   • Polycythaemia vera (PV)
   • Essential thrombocythaemia (ET)
   • Chronic myeloproliferative disease, unclassifiable
2. Myelodysplastic/Myeloproliferative Diseases:
   • Chronic myelomonocytic leukemia (CMML)
3. Myelodysplastic Syndrome (MDS):
   • Refractory anemia (RA)
   • Refractory anemia with ring sideroblasts (RARS)
   • Refractory cytopenia with multilineage dysplasia (RCMD)
   • RCMD with ringed sideroblasts (RCMDRS)
   • Refractory anemia with excess blasts (RAEB1)
   • RAEB2
   • Myelodysplastic syndrome unclassified (MDSU)
   • MDS with isolated del 5q
4. Acute Myeloid Leukaemia (AML):
   • AML with recurrent cytogenetic abnormalities
     • AML with t(8;21) (q22;q22)
     • AML with abnormal bone marrow eosinophils {inv (16) (p13q22)}
     • Acute promyelocytic leukaemia {t(15;17) (q22;q12)}
     • 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 categorized
     • AML, minimally differentiated
     • AML without maturation
     • AML with maturation
     • Acute myelomonocytic leukemia (AMML)
     • Acute monoblastic and monocytic leukemia
     • Acute erythroid leukemia
     • Acute megakaryocytic leukemia
     • Acute basophilic leukemia
     • Acute panmyelosis with myelofibrosis
     • Myeloid sarcoma
5. Acute Biphenotypic Leukaemia

Chronic Myeloid Leukemia:

• Etiology:
  • It is a myeloproliferative disorder.
  • Occurs as a result of the malignant transformation of pluripotent stem cells leading to the accumulation of a large number of immature leukocytes in the blood.
  • Radiation exposure and genetic factors have been implicated in the development of CML

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 hematomas may occur.
• Visual disturbance, neurologic manifestations.
• Juvenile CML is more often associated with lymph node enlargement than splenomegaly.

Peripheral Blood Picture:

1. Leucocyte count is elevated often > 1,00,000 cells/1.
2. Circulating cells are predominantly neutrophils, metamyelocytes, and myelocytes but basophils and eosinophils are also prominent.
3. The typical finding is an increased number of platelets (thrombocytosis).
4. Anaemia is usually of moderate degree and is normocytic, normochromic in type. Normoblasts may be present occasionally.
5. A small portion of myeloblasts usually <5% are seen.

Bone marrow examination:

1. Cellularity Hyper is cellular with total/partial replacement of fat spaces by proliferating myeloid cells.
2. Myeloid cells Myeloblasts are only slightly increased.
3. Erythropoiesis Normoblasts but there is a reduction in erythropoietic cells.
4. Megakaryocytes are Conspicuous but are usually smaller in size than normal.
5. Increase in number of phagocytes.

Chronic Myeloid Leukemia Management:

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

Question 8. Mention various types of diagnostic criteria and complications of leukemia, outline the significance of the system disorder in dental practice

Answer:

Complications of Leukemia:

• Infections
• Clogging in blood vessels
• Stroke
• Impaired bodily functions
• Development of other cancers like
  • Kaposi sarcoma
  • Melanoma
  • Lung cancer
  • Stomach cancer
  • Throat cancer
• Death

Dental Significance:

Question 9. Describe etiological factors, clinical features, and management of eosinophilia

Answer:

Eosinophilia

An increase in the number of eosinophilic leukocytes is referred to as eosinophilia.

The causes of eosinophilia are as follows:

1. Allergic disorders: Bronchial asthma, urticaria, drug hypersensitivity
2. Parasitic infestations: Trichinosis, echinococcosis, intestinal parasitism.
3. Skin diseases: Pemphigus, dermatitis herpetiformis, erythema parasitism.
4. Certain malignancies: Hodgkin’s disease and some non-Hodgkin’s lymphomas.
5. pulmonary infiltration which is eosinophilia syndrome
6. Irradiation.
7. Miscellaneous disorders: Sarcoidosis, rheumatoid arthritis, polyarteritis nodosa.

Eosinophilia Clinical Features:

• Dyspnoea
• Orthopnoea
• Wheezing
• Cough with mucoid expectoration
• Chest pain

Eosinophilia Management:

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

Question 10. Describe oral manifestations of hematological disorders. How would you treat a case of agranulocytosis

Answer:

Hematological Disorders:

1. Disorders due to vascular disorders:
   • OslerWeberRendu disease
   • Inherited disorders of the connective tissue matrix
   • Acquired vascular bleeding disorders
2. Disorders due to platelet disorders:
   • Thrombocytopenia
   • Thrombocytosis
   • Disorders of platelet functions
3. Coagulation disorders:
   • Hemophilia A
   • Hemophilia B
   • Von Willebrand disease
4. Disorders due to Fibrinolytic defects
5. Disseminated intravascular coagulation, DIC

Hematological Disorders Oral Manifestations:

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

Treatment of Agranulocytosis:

• Removal of the offending agent
• Transfusion of red cell constituent when hemoglobin is less than 10 gm/dl
• Antibiotics to control septicemia
• Combination of drugs
• Administration of granulocyte-macrophage colony-stimulating factors
• Dental management for ulcers5% dyclonine and 5% Benadryl mixed with magnesium hydroxide or Kaolin with pectin

Question 11. How will you investigate a case of bleeding diathesis? Mention some of the dental considerations

Answer:

Bleeding Diathesis Investigations:

1. Investigations of disordered vascular hemostasis
   • Bleeding time
     • It is based on the principle of formation of a 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 appearing in the next 5 minutes in a 3 cm² area over the cubital fossa is counted
     • The presence 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 methods
       • Normal value 49 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 of prothrombin time measures the extrinsic system factor VTT 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

Bleeding Diathesis Dental Considerations:

• 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 antifibrinolytic 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 12. Classify bleeding and coagulation disorders. Write clinical features, diagnosis, complications, and management of hemophilia.

Answer:

Bleeding and Coagulation Disorders Classification:

1. Disorders due to vascular disorders
   • OslerWeberRendu disease
   • Inherited disorders of the connective tissue matrix
   • Acquired vascular bleeding disorders
2. Disorders due to platelet disorders
   • Thrombocytopenia
   • Thrombocytosis
   • Disorders of platelet functions
3. Coagulation disorders
   • Hemophilia A
   • Hemophilia B
   • Von Willebrand disease
4. Disorders due to fibrinolytic defects
5. Disseminated intravascular coagulation, DIC

Hemophilia A:

• It is an inherited disorder of factor 8 deficiency

Hemophilia A Clinical Features:

• Initially diagnosed in infancy Males are commonly affected
• Characterized by easy bruising
• Prolonged bleeding after trauma
• Bleeding into subcutaneous tissue
• Hematoma formation
• Epistaxis
• Gastric hemorrhage
• Recurrent hemarthrosis
• Osteoporosis
• Intracranial hemorrhage
• Hematuria

Hemophilia A Oral Manifestations:

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

Hemophilia A Diagnosis:

• Bleeding time normal
• Prothrombin time normal
• Platelet count normal
• Activated partial thromboplastin time prolonged
• Specific factor 8 assay

Complications:

• Excessive blood loss
• Bleeding in brain
• Long-term joint problems
• Abnormal thrombosis and clot formation

Management:

• Replacement therapy by the use of fresh frozen plasma
• Administration of factor 8
• Prevention of complications by the use of a synthetic analog of antidiuretic ldeamino8darginine vasopressin
• Genetic counseling to prevent inheritance

Question 13. Describe etiopathogenesis, clinical features, investigations, and management of hemophilia B

Answer:

Hemophilia B

Etiology:

• It is an inherited 10linked disease due to a reduction in plasma factor 9 level
• It is due to aberration in the factor 9 gene

Hemophilia B Clinical Features:

• Prolonged bleeding during circumcision
• Prolonged bleeding after surgical procedures
• Prolonged bleeding from cuts
• Excessive bruising
• Excessive and prolonged epistaxis
• Blood in urine and feces
• Internal bleeding causing pain and swelling
• Bleeding in the skull after childbirth
• Spontaneous bleeding

Hemophilia B Investigations:

• Factor 9 test
• Activated partial thromboplastin time
• Prothrombin time
• Fibrinogen test

Hemophilia B Management:

• Factor 9 injections
• Application of desmopressin acetate to small wounds