2F Hypoproliferative Anemias

Dr. Kua | March 29, 2017

FINALS QUIZ 1

PANCYTOPENIA Hb= 124 Hct= 35% WBC= 2.9 N= 24% L=

Reduction in all (3) blood cells elements in the peripheral 38% M= 37%? E= 1%
blood (circulation) Platelet count= 23,000
o Hemoglobin RBC= normocytic, normochromic, 6 NRBC
o Total WBC Diagnosis: Acute Promyelocytic Leukemia
o Platelet (Thrompocytopenia)
Bicytopenia, monocytopenia CASE 4: PB, 67 F, B+
o Evolving PANCYTOPENIA, aplastic anemia?
Recently transfused with 2 u PRBC,
Normal CBC Parameters 10 u PC (Platelet concentrate)
PARAMETERS NORMAL VALUES Abdominal US= Mild hepatosplenomegaly
Hb Male: 140-160 gm/L In Aplastic Anemia, no organomegalies
Female: 120-140 gm/L
Hct Male: 0.44-0.55 CASE 5: JU, 25 F, O+
Female: 0.35-0.47 Gum bleed, easy bruises, profuse menstrual flow, pallor
WBC 5.0 10.0 x 109/L Hb=40 Hct=12% WBC=2.8 N=49
Neutrophils/Lympho 0.60/0,40 L=50 M=1
Mono, eosino, baso 0-0.40 Platelet count= 29.0
Platelet count 140.0 450.0 x 109/L BMA = Aplastic anemia
RBC morphology Normocytic, normochromic 10 u platelet concentrate
5 u PRBC
Typical CBC in Aplastic Anemia (Pancytopenia) IV hydrocortisone, folic

CASE 6: AV, 56 M, O+
Confined for flu-like symptoms; persistent
granulocytopenia
Jan. 13 Feb. 1 Mar. 19 Mar. 24
H/H 146/43 157/45 147/42 133/40
Usual parameters: WBC 2.86 2.6 1.5 2.0
o Low hemoglobin N/L 32/47 53/34 53/41 51/43
o Variable degree of leukopenia - Total WBC count Mono 18 13 6 6
o Thrombocytopenia Platelet 181 270
o Differential count: Neutropenia/Granulocytopenia Acute Leukemia
that generates fever or infection, not the true/relative
lymphocytosis. Clinically important value:
APPROACH TO PANCYTOPENIA
Neutropenia.

CASE 1: MB, 61 M
Pallor
Hb= 67 Hct= 20% WBC= 3.5 N= 43% L= 57%
Platelet count= 102,000
RBC= normocytic, normochromic
Pancytopenia

CASE 2: MC, 87 F
Pancytopenic CBC regardless of the clinical profile
Pallor
Usually, anemia is the dominant problem.
Hb= 54 Hct= 16% WBC= 3.0 N= 27% L= 77%
M=2% E=2%
Platelet count= 62,000
RBC= normocytic, normochromic
Pancytopenia

CASE 3: BG, 43 M
Fever, blood per expectorate
Benign Causes of Pancytopenia (Hematologic) o Decreased CD34 (stem cell marker) cells
Aplastic anemia o Replacement by fatty tissue
o Idiopathic / Secondary / Hereditary Bone marrow failure
Folate, B12 deficiency Donor hematopoietic stem cell transplant
PNH
Hypersplenism Clinical Presentations
Autoimmune disorders Asymptomatic/incidental
Systemic infections o CBC, PBS
Chronic liver disease Anemia, pallor alone (with or without bleeding)
o (+/-) splenomegaly (hypersplenism) Bleeding alone
Drug-induced Fever, infection alone
Combination
Malignant Causes of Pancytopenia o Pallor, bleeding, infection/fever
Blood malignancies
o Leukemias Clinical Symptomatology
o Lymphomas Mild, moderate, severe presentation, therefore observe
o Plasma cell dyscrasias (Myeloma) for years Reassure patient
Metastatic tumors to the marrow NO hepatosplenomegaly
(Myelophthisis) NO adenopathies
Drug-induced Insidious clinical onset (Weeks to months)
Acute presentation in some cases
APLASTIC ANEMIA
Epidemiology CBC/PBS Findings
Incidence Low Hb, Hct
o Rare among Caucasians RBC = Normocytic, normochromic
o Common among Asians Reticulocytopenia Not usually done, usually normal
Europe No polychromatophilia, no nucleated RBC, no
o 2 per million persons annually leucoerythro-blastic picture
Thailand and China Leucopenia/neutropenia
o 5 7 per million o Relative lymphocytosis
Equal male: female incidence o Neutropenia will make the problem, not the
Biphasic age distribution lymphocytes
o Older children and young adults No immature red or white cells
o Elderly Low platelet count
Increased frequency in ages over
60 (whites) Laboratory Findings
Hemostatic changes
Etiopathogenesis Bleeding time = normal to prolonged
Idiopathic (Primary) Coagulation tests = within normal
o Majority (Over 90%) Clot retraction = poor to none (not usually done)
Secondary
o Drug-induced, Chemicals (Benzene) Marrow Aspirate Findings
o Post-infectious, Immunologic event Bone marrow (aspirate/biopsy) study
o Radiation o Dry tap, Diluted marrow blood
o Hereditary o Marrow biopsy needed 2F MEDICINE 2: Hypoproliferative Anemia
o Fanconis anemia Hypocellular
o Others o Fatty
o Myelodysplasia, PNH o Prominence of fibrous connective tissue network
o Herbicides and pesticides NOT proven o Decreased myeloid, erythroid & megakaryocyte
o Relative lymphocytosis
Pathophysiology o No granuloma, blast cells, metastatic cells,
Severe damage to hematopoietic stem cell compartment megaloblastosis

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Marrow Core Biopsy Therapeutic Options
Hypocellular pattern Immunosuppressive agents
Increased fatty spaces Androgens
Decreased Supportive measures- BT components
myeloid elements Hematopoietic stem cell transplant for severe cases

Normal marrow smears (low power) Immunosuppressive Agents

o Fat space with a lot of cells Steroids oral prednisone / IV hydrocortisone
o 50% Cells and 50% Fat o Partial vs. complete responses
o Availability, cost
o Side effects
Anti-thymocyte globulin (ATG)
o Responses
o Availability, cost
o Side effects
o Steroids
o Anti-thymocyte globulin, CY A
Normocellular Cyclosporine A

Androgens
Responses
Availability, cost
Hypocellular Side effects especially when given to females or children,
discuss with the parents
Cellular to hypercellular; no fatty spaces
Supportive Measures
Component transfusions for platelet thrombocytopenia
Growth factors - GCSF, EPO- for
Folic acid supplement
Contraception for regulation of uterine endometrial
Metastatic cells (in clumps): Non-hematopoetic activity
malignancies Antibiotics for infectious events

Removal of suspected cause

No treatment needed if there are no symptoms. Treating
asymptomatic patients will just create a new problems due
to the side effects of drugs.

Diagnosis Prognosis
PB pancytopenia Mild, moderate cases
o No immature WBC/RBC
Spontaneous recovery = few
o Normal RBC morphology
Severe aplastic anemia
Marrow hypocellularity
2F MEDICINE 2: Hypoproliferative Anemia
o ANC < 500 cells/mm3
o No cellular histologic changes
o Platelet count < 20,000/mm3
Pancytopenia with hypercellular marrow (BENIGN) o Survival at 1 year after diagnosis 20%
o PNH
o Systemic diseases (SLE, hypersplenism, B12 and folate BMT is the Only Curative Option for Severe AA
deficiency, severe infection, TB)
Everything else is just palliative
Pancytopenia with hypercellular marrow (MALIGNANCY)
Bone marrow transplantation
o Leukemias
o Hematopoietic stem cell transplantation (HSCT)
o Lymphomas
o HSC IV infusion
o MDS (myelodysplatic syndrome)
o Allogeneic vs. syngeneic
o Metastatic
Allogeneic: HLA compatible (e.g. Twins)
Syngeneic: Willing donors
Diagnosis of aplastic anemia is partly a diagnosis by o Curative in over 80% of cases
exclusion

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 SUMMARY
Asymptomatic
o Abnormal CBC
Anemia/pallor, bleeding, fever/infection combination-
due to thromboembolism
Chronic course
Very Variable degree of clinical and hematologic severity
Pancytopenia
o Bicytopenia
o Monocytopenia
Aplastic anemia or not
o Entertain secondary causes
o Possible reversibility
Benign vs. malignancy
o Benign diseases
Folic acid, B12 deficiency
Hemolytic disorder
Drug-induced, chemicals, radiation
Post-infectious
Immunologic diseases
Hereditary
o Malignancy
Blood leukemias, lymphomas, myeloma, MDS
Metastatic breast, lung, prostate, GIT, etc
Marrow evaluation
o Marrow aspiration
o Marrow core biopsy
o Hypoplasia
o No other abnormalities
Palliative symptomatic treatment
o Steroids & immunosuppressive agents
o Folic acid
o Androgens
o Component transfusion
Severe aplastic anemia
o Stem cell transplant can be curative in many cases

Cruz, G. I Disu

2F MEDICINE 2: Hypoproliferative Anemia

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