Hematological Disease Part 1 PDF

Hematological Disease “Dr.Gillan El-Kimary” Process Of Hematopoiesis Hematopoietic stem cells located in the adult bone marrow are the progenitors from which all blood cell lineages arise There are three important factors in the formation of RBCs 1. Iron : a crucial...

Hematological Disease “Dr.Gillan El-Kimary” Process Of Hematopoiesis Hematopoietic stem cells located in the adult bone marrow are the progenitors from which all blood cell lineages arise There are three important factors in the formation of RBCs 1. Iron : a crucial component of the hemoglobin molecule 2. Erythropoietin: secreted by kidneys, promotes the formation of RBCs in bone marrow 3. Vitamin B12 : important for RBCs maturation. RBCs 120 days Platelets 5-10 days Neutrophils 6-8 hours Lymphocytes Many years Senescent cells are sequestrated by three main organs: Spleen , Liver and Bone Marrow through phagocytosis by macrophages Mean Corpuscular Volume (MCV) It is the measure of average volume (size) of RBCs Mean Corpuscular Hemoglobin (MCH) It is the measurement of the average weight of Hb in individual RBC Mean Corpuscular Hemoglobin concentration (MCHC) It is the average concentration of Hb per unit volume Hematocrit (HCT) Percentage of a sample of whole blood occupied by intact RBCs 1  Aplastic Anemia (bone marrow aplasia) Normocytic Normochromic Anemia A rare heterogenous and potentially fatal blood dyscrasia characterized by pancytopenia & hypocellular bone marrow Etiology Idiopathic Inherited : Fanconi Anemia. Acquired : 2ry Bone Marrow toxicity--> Due to; viruses, radiation, toxins or drugs (as sulfonamides, anti-rheumatic & chloramphenicol). Chemicals. Clinical Features o Anemia General manifestations of anemia. o Leukopenia Increased susceptibility to infection. (candidiasis and viral infection) o Thrombocytopenia Purpura and bleeding ,epistaxis or gingival bleeding. Oral manifestations o Pallor, gingival bleeding, petechiae & ecchymosis. o Ulcers as in neutropenia, surrounded by little erythema. o Herpetic infections and recurrent candidiasis. o Advanced cases resembles acute leukemia Diagnosis A hypocellular BM along with evidence of depression of at least blood cell lineages is required to establish the diagnosis Further cytogenetic testing is useful to distinguish inherited from acquired forms of AA. Bone marrow karyotyping. Lab. Findings (CBC) MCV normal MCHC normal 2  Hb RBC’s Platelets decreased WBC s The most serious fatal complication of AA is severe viral, fungal, and bacterial infections. (Prophylactic Antibiotics) Oral Health Considerations ✓ Patients with non-severe AA can generally tolerate routine care. ✓ Severe AA cases are at a high risk for hemorrhagic and infectious events. ( should be managed in a hospital setting) ✓ For patients who are severely neutropenic (neutrophil count 52% males, >48% females) OR elevated hemoglobin (Hb) level (185 mg/dL for males, >165 mg/dL for females) 3 Polycythemia Vera (PV) - Unknown etiology, may be genetic.(JAK2 gene mutation ) - Peak incidence in 6th decade. - RBCs increase 6-12 million/mm - Increase blood viscosity & thrombosis Oral Manifestations - Erythema (red–purple color) of mucosa - Glossitis, and erythematous, edematous gingiva - Gingival bleeding tendency - Varicosis in the ventral tongue - Petechiae, ecchymosis of oral mucosa. Oral Health Considerations CBC is essential before Rx. Reduce Hb level below 16g/dl and hematocrit below 52% Delivery of routine dental care for the well-controlled patient with PV likely incurs minimal risk Poorly controlled patients are at an increased risk for both thrombotic & hemorrhagic due to blood hyper-viscosity 4  Medical consultation to determine the current patient status should be obtained Diagnosis 1) Hb > 18.5g/dl for males , > 16.5 for females OR other evidence of increased red-cell mass 2) JAK2 gene mutation 3) BM showing hypercellularity Anemia Defined as a lower than normal Hb concentration (