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Title: Must Knows of Hematologic Diseases
Description: Concepts on Hematologic Diseases 1. Acute and chronic ATP 2. Folic acid and Vit B12 Deficiency 3. Alpha and Beta Thalassemia 4. Hodgkins's and Non-Hodgkins Lymphoma 5. Lymphocytic and Myelogenous Leukemia 6. Aplastic anemia and Paroxysmal Nocturnal Hemoglobinuria etc...
Description: Concepts on Hematologic Diseases 1. Acute and chronic ATP 2. Folic acid and Vit B12 Deficiency 3. Alpha and Beta Thalassemia 4. Hodgkins's and Non-Hodgkins Lymphoma 5. Lymphocytic and Myelogenous Leukemia 6. Aplastic anemia and Paroxysmal Nocturnal Hemoglobinuria etc...
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MUST KNOWS ON HEMATOLOGY
HEALTH AND MEDICAL
John Lorenz Beleno, RM, RN, MD
Acute ATP
Mainly a disease of children
Cross reaction antibodies to non-specific platelet membrane proteins
Chronic ATP
Frequently seen in young women
(+) autoreactive antibodies against platelet GpIIb-IIIA complex membrane
Precedent febrile illness or URTI
Folic Acid Deficiency Anemia
True chronic alcoholism
Concomitant with chronic hemolytic disorder
Source
Absorption
Metabolism
Folic Acid Metabolism
Plants and vegetables
Proximal intestine
Active cellular form: Methyl tetrahydrofolate
Seen in
Chronic alcoholics
Alpha Thalassemia
May cause hydrops fetalis
Etiology: one or more globin genes in Chromosome 16
Vitamin B12 Deficiency Anemia
MC cause: malabsorption syndrome
Aka: Pernicious anemia
Severe form: vibratory sense abnormality
Etiology: lack of gastric intrinsic factor
Vitamin B12 Metabolism
Meat and dairy products
Distal ileum
Requires intrinsic factor,
Co-factor in methionine synthesis
Malabsorption syndromes
Beta Thalassemia
Pathology: Persistence of fetal hemoglobin
Etiology: globin mRNA processing
(+) Bart’s hemoglobin or HbH
Hodgkin’s Lymphoma
(+) Reed-Sternberg cells
Types: lymphocyte predominance, lymphocytic depletion, nodular
sclerosis, mixed cellularity
Single most important prognostic marker: Stage of disease at diagnosis
Non-hodgkin’s Lymphoma
Aka malignant lymphoma or lymphocytic lymphoma
Types: well-differentiated, poorly differentiated; diffuse, follicular; small-cell,
large-cell
Risk: transplant subjects, HIV infection
Extra-nodal presentation
Acute Lymphocytic Leukemia
MC in children less than 10 years old
Etiology: viral in some cases
Presentation: adenopathies, splenomegaly
Tx: Vincristine, prednisone, doxorubicin, L-asparginase
FAB subtypes: L1, LL2, L3
Acute Myelogenous Leukemia
MC in young adults and elderly
Tx: 7 + 3 (cytarabine + doxorubicin), L-asparginase
FAB subtypes: M1 to M7
Malignant degeneration of multipotent stem cells
Blast cells > 20%
Chronic Lymphocytic Leukemia
Rarest type of leukemia among Asians
Mature lymphocytosis
Chronic Myelogenous Leukemia
Most common and highly prevalent among adult Caucasians
Marked leukocytosis with shift to the left
Mainly affects young adults and elderly population
Asymptomatic, painless adenopathy
Can transform into acute lymphoid or myeloid leukemia during blastic crisis
Philadelphia chromosome (+) in > 90%, translocation abnormality: 9 and 22
Abelson oncogene overexpression
DOC: prednisone, chlorambucil, cyclophosphamide, fludarabine
DOC: Oral Imatinib and other 2nd generation t-kinase inhibitors
Tx: bone marrow transplant curative
Aplastic Anemia
Damage to CD34+ stem cells
Tx: Packed RBC preferred
(then: bone marrow transplantation)
Extravascular hemolytic process
Elevated total bilirubin, unconjugated bilirubin fraction, urobilinogen, fecal
urobilinogen
Paroxysmal Nocturnal Hemoglobinuria
Deficiency of CD 55 or CD 59 membrane proteins in the affected red cells
Anemia is due to complement-mediated lysis
Tx: Washed RBC preparation preferred
Intravascular hemolytic process
Elevated plasma carbon monoxide gas level
Low serum haptoglobin, low serum hemoprexin, presence of
methemalbumin (severe hemolysis)
Polycythemia vera
Erythrocytosis + splenomegaly
Congenital heart disease, chronic pulmonary
disease, smoking must be ruled out
Primary Myelofibrosis
“teardrop” red cells in the PBS
Anemia and splenomegaly
Essential Thrombocytopenia
Bleeding and/or thrombosis
Major Hemoglobin
(HbA1)
2 alpha + 2 beta globin units
(A + B)
Main Hgb variant present in young children and
adults
Minor Hemoglobin
(HbA2)
2 alpha + 2 delta globin units
(A + D)
Fetal hemoglobin
(HbF)
2 alpha + 2 gamma globin units
(A + C)
High concentration in infants
< 6 mos old
Normally present in trace to negligible amount in
adult blood sample
Hereditary Spherocytosis
(+) Osmotic fragility test
Splenectomy curative
Osmotic Fragility Test
G6PD enzyme deficiency hemolytic
anemia
Normocytic, normochromic
Medication intake triggers episodic
hemolytic anemia
Hemoglobin Electrophoresis
Hereditary spherocytosis
Thalassemias
Packed Red Cell
For symptomatic chronic
anemia without associated
hemorrhage
Frozen Plasma
Used to control bleeding in a
chronic liver cirrhotic patient
Thalassemia
Sickle Cell Anemia
Hypochromic, microcytic
Deficient cell membrane-associated
spectrin in many cases
Ham’s Test
Paroxysmal Nocturnal
Hemoglobin
Cryoprecipitate
Controls bleeding in a known
hemophilia A patient
Coomb’s Test
Autoimmune Hemolytic
Anemia
Fresh Whole Blood
Neonatal exchange
transfusion
Due to single DNA base mutation in
the 6th beta globin gene triplet code
Frequently associated with venous
stasis and leg ulcers
Red Cell Metabolic Enzyme
Assay
G6PD deficiency hemolytic
anemia
Platelet Concentrate
Controls bleeding in any
thrombocytopenic condition
Title: Must Knows of Hematologic Diseases
Description: Concepts on Hematologic Diseases 1. Acute and chronic ATP 2. Folic acid and Vit B12 Deficiency 3. Alpha and Beta Thalassemia 4. Hodgkins's and Non-Hodgkins Lymphoma 5. Lymphocytic and Myelogenous Leukemia 6. Aplastic anemia and Paroxysmal Nocturnal Hemoglobinuria etc...
Description: Concepts on Hematologic Diseases 1. Acute and chronic ATP 2. Folic acid and Vit B12 Deficiency 3. Alpha and Beta Thalassemia 4. Hodgkins's and Non-Hodgkins Lymphoma 5. Lymphocytic and Myelogenous Leukemia 6. Aplastic anemia and Paroxysmal Nocturnal Hemoglobinuria etc...