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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...

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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...