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Hematology Problems

Leukemia: Leukemia is a type of cancer found in the blood and bone marrow and is caused by the rapid production of abnormal white blood cells.
These abnormal white blood cells are not able to fight infection and impair the ability of the bone marrow to produce red blood cells and platelets

Unregulated proliferation of one particular type of leukocytes be it granulocytes; monocytes; lymphocytes, erythrocytes, monocytes or
megakaryocytes

Defect originates in the hemapoetic stem cells (Myeloid or lymphoid)

Acute leukemia; Onset is rapid, occurring with a few weeks, Leukocyte development are halted at the blast phase so that most leukocytes are
undifferentiated or are blast This condition progresses rapidly , death occurs within weeks to months without aggressive treatment.

In chronic leukemia symptoms evolve over a period of months to years and him majority of leukocytes produced are mature. The disease
process is slower and can extend for years

Type Defect Incidence Prognosis Diagnosis Manifestations Medical mgt

Acute Stem cells that Affects all ages. Younger may ↓erythrocytes Neutropenia: fever; Aggressive chemo
myeloid differentiates Peaks @ age 60 survive for 5 ↓platelets infection induction therapy
Leukemia into all myeloid years or more Leukocyte count Anemia: weakness & Supportive therapy
(AML) cells Most common may be ↓, ↑or fatigue (epogen/neupogen)
non-lymphocytic Cells of older normal but % of Pain: due enlarged liver
leukemia. pts are more normal cells is ↓ & spleen BMTor SCT
undifferentiate Bone pain Assess for graft vs.
d Prognosis Bleeding tendencies host disease
worst Hyperplasia of bones
&gums
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Type Defect Incidence Prognosis Diagnosis Manifestations Medical Mgt

Chronic Mutation in the Incidence Blast stage = 6 Translocation of 3 stages: Acute phase tx
Myeloid myeloid stem increases with months chromosome 22  Chronic resembles the tx for
leukemia cells age Leukocytosis-  Transformation AML ( BMT and
(CML) Translocation of Mean age of Chronic stage = increased level of  Accelerated or or PBST)
chromosome 22 occurrence55-60 3-5 years leukocytes(WBC blast stage
(Philadelphia ) Mostly older ) in the Meds
to chromosome adults circulation Initially may be **Gleevec
9 Uncommon in asymptomatic (antacids and
people under 20 Leukocyte count grapefruit juice
Normal ↑100,000 (leukocytosis). block the
myeloid cells Most common SOB and or confusion absorption)
continue to be form of leukemia
produced but in US Enlargement of marrow Imatinib (appears
there is a producing organs more useful during
pathologic (spleen , bone marrow) = the chronic stage)
increase in the pain
production of Hepatomegaly
blast cells. Wt loss; anorexia

Acute Uncontrolled Common in ↓leukocytes or Immature lymphocytes Goal: Complete

Lymphocyti proliferation of children 5-year can also be ↑ (% proliferate bone marrow remission
c Leukemia immature Peaks @ age 4 survival of immature cells and decrease the Tx is Complex:
(ALL) lymphoid stem Rarely occur 80% children will be high) development of normal Induction therapy
cells (precursor after age 15 40% adult ↓erythrocytes myeloid cells. Lymphoid cells are
to T lymphocyte Affects more ↓platelets Infiltration into other sensitive to
25% boys than girls organs are more common Steroids and
B lymphocyte Frequently invades the Vinca Alkaloids.
75%) CNS Cranial
irradiation &
May be found in the Intrathecal
Central nervous system; chemotherapy
also, testes and breasts.
Maintenance
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Pain in liver, spleen & phase lower dose

bone chemo for 3 years

Headache ; vomiting Steroids, Imatinab,

BMT, SCT
Type Defect Incidence Prognosis Diagnosis Manifestations Medical mgt

Chronic Malignant clone Common in Average Normal or Early: Early stage No Tx-
Lymphocyti of B lymphocyte older adults survival age: ↓erythrocytes and ↑lymphocyte count monitor
c Leukemia (T lymphocyte 2/3 are > 60years Early stage platelets Lymphadenopathy-
(CLL) CLL is rare) =14yrs Swollen painful nodes; Late stage: Begin
Affects more Late stage = Enlarged liver and spleen treatment
*Most of men 2ys Fludura;
leukemic cells of Survival for Later stage: corticosteroids;
CLL are mature, male is less Thrombocytopenia Chlorabucil
(may have than female Auto-immune (leukeran)
escaped or complications can occur
resisted at any stage. Cytoxan ;
apoptosis(cell B symptoms: Night Vincristine
death) sweats, unintentional wt Bactrim
loss; infections prophylaxis
****Nursing Care: see text*****
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Lymphoma

Neoplasms of lymphoid cell origination

Usually originate in the lymph nodes but can involve lymphoid tissues of the spleen, GI tract, liver or bone marrow.

Classified according to the level of differentiation and the origin of the predominant malignant cells.

Classified broadly into two categories:

 Hodgkins
 Non -Hodgkin’s.

Type Defect Incidence Prognosis Diagnosis Manifestations Medical mgt

Hodgkins Cause unknown. Rare. With Biopsy for + Reed Unilateral, painless Goal: Cure
Lymphom Thought to be More common treatment has Sternberg cell. enlargement of lymph
a an immature in men an impressive node on neck. Treatment:
lymphoid cell: cure rate Mediasternal mass on dependent on stage
Reed Age: Peaks in X-ray. Sx: related to Traditionally
Sternberg cell early 20’s and Assess for B compression of organs treated with
after age 50 symptoms involved laporotomy and
Thought to be PET scan; CT of Compression of trachea; radiation
viral etiology. Risk: Pts chest, abd and or Cough ; pleural effusion ; Currently tx with
receiving pelvis abdominal pain; 22-4 months
Unicentric in chronic Bone marrow biopsy Pruritus; Herpes Zoster chemo followed by
origin immune- Lab: EST, Liver & Severe pain on ingestion radiation
suppressive Renal studies of alcohol; anemia; B Reoccurrences
Spreads by therapy (renal symptoms; normal or responds well to
contiguous transplants); slightly decreased secondary chemo
extension into woodworkers platelet count; decrease and radiation
the lymph nodes Military- skin sensitivity test. followed by
exposure to All organs are vulnerable autologous BMT
agent orange to invasion or stem cell
transplant

Type Defect Incidence Prognosis Diagnosis Manifestations Medical mgt

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Non Heterogeneous 7th most Varies Highly complex Highly variable Based on
Hodgkins group of cancers common cancer 33% cure rate. CT; PET; Bone symptoms. classification, 1.
Lymphom that originate in US marrow biopsy CNS Lymphadenopathy is Stage of disease,
a from neo-plastic Age: Ave age fluid analysis most common symptom 2. Previous
growth of 50-60 and (66% ) treatment and
lymphoid Incidence B symptoms (33%) 3. Patients’
tissues increases with Less aggressive forms tolerance level.
each decade of can wax and wane
Most involve B life. Asymptomatic in early Bone marrow
lymphocyte stage. transplant& stem
5% involve T Lymphadenopathy in cell transplant
lymphocyte stage 3 -4. may be considered
Lymph masses can for younger
Unpredictable compromise organ patient.
spread. functions e.g. respiratory,
Invades spleen CNS; urinary. Chemo
multiple lymph Radiation: If the
node sites disease is not
Localized aggressive
disease radiation alone
uncommon may be needed.

Lifetime
screening
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Multiple Myeloma

Type Defect Incidence Prognosis Diagnosis Manifestations Medical mgt

Multiple Malignancy of Ave age 65-70 No cure Lytic lesions and Bone destruction Chemo
Myeloma most mature B yrs Median Osteoporosis on X-ray 2/3 of pts have bone pain Corticosteroids
lymphocytes i.e. Slightly more survival time: (back and ribs) such as
Plasma cells common in male 3-5 years. ↑Monoclonal protein Pain on movement. Dexamethasone
with destruction than female in urine serum Pain decreases with rest Thalidomide
of bone. (note it 2x more in Cause of death M-Protein serves as a Less pain on awakening Velcade
is not classified Black than white usually marker to monitor More pain as the day
as a infection disease progression progresses. Radiation to
lymphoma)* Elevated total protein. Hypercalcemia : (severe strengthen the
These disease Bench Jones protein thirst; dehydration; bone; relieve pain;
cell produces constipation; AMS; and reduce tumor
nonfunctional Anemia or confusion; coma) size.
immunoglobulin hypercalcemia Renal failure Vertebroplasty to
Presence of plasma Anemia ( fatigue, treat vertebral
Angiogenesis , sheets weakness) fracture or
the malignant ↓Platelets & ↓leukocytes compression.
cells stimulate Confirmation: bone Infection Plasmapheresis to
the creation of marrow biopsy treat viscosity
new blood Infiltration or invasion of
vessels to other tissues BMT and stem cell
enhance their (plasmacytomas) transplant to
growth Symptoms of anemia, extend remission
fatigue weakness.
Increase serum viscosity
Increase risk for
bleeding
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Coagulation disorders

Type Defect Incidence Prognosis Diagnosis Manifestations Medical mgt

Primary Stem cell Women 2x Survival R/o other Initially:

(Essential) disorder more than Improved disorders. Asymptomatic Highly controversial.
Polycythemia within the men with CBC shows large Later: Hemorrhage or Complications occur at
bone marrow Median age: treatment and abnormal vascular occlusion. platelets that exceed
↑in platelet 65-70yrs platelets. Persistent Thrombosis can be 1.500,000.
production platelet of 600,000 venous or arterial.
with count or greater. Painful burning, Low dose ASA for
↑600/000 warmth and redness. Younger pts with no
Headaches ; contraindications :
Size numbness, visual
abnormal disturbances Platelet pheresis
Occasionally (diplopia)
↑erythrocyte TIA; DVT and Older pts: More
s and /or pulmonary embolism aggressive treatment:
↑leukocytes Seizures Strokes, MI Chemo agent
Slight enlargement of Hydroxyurea is used to
Rarely the spleen lower platelet count.
evolves into Interferon –alfa-2b TIW.
leukemia Bleeding (eccymosis,
hematomas; epitaxis
& gum bleeding)
Major bleeding occurs
at platelet count
greater than
1,500,000.
GI bleed.
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Type Defect Incidence Prognosis Diagnosis Manifestations Medical mgt

Idiopathic Autoimmune Common in Diagnosed by Asymptomatic Not initiated unless

Thrombocytopeni disorder children and exclusion Low platelet count is bleeding becomes life
a Purpura (ITP) characterized young Platelets < 20.000 an incidental finding threatening (10,000)
by a women ↑Megakaryocytes Easy bruising; heavy
destruction Acute: in bone marrow. menses; petichae on Goal***************
of normal Mostly in May have + trunk and or safe platelet count.
platelets by children H.pylori extremities. Stop any medication
an unknown appears 1-4 Pts with Simple associated with ITP
stimulus. weeks after The platelets are bruising tend to have ( e.g. sulfa drugs)
Anti platelets viral illness young and fully less complication than
antibodies Remission functional. those with wet Immunosuppressant
bind to the occurs They adhere to purpura. therapy –Imuran
platelets. spontaneousl themselves and to Pts with wet purpura Corticosteroids:
RES system y with 6 the endothelial is at greatest risk for Prednisone;
ingests the months tissues so that intracranial bleeding Dexamethasone
platelets. spontaneous despite low platelet IVIG
The body Chronic bleeding count Surgery: Splenectomy
compensates Diagnosed by Complications;
by increasing exclusion Osteoporosis; Monitor Bone density
platelets proximal wasting; Calcium & Vit D
production. cataract; Dental caries replacement
Can be
induced by
sulfa drugs,
viral
infection
lupus;
pregnancy.
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Factor Deficiency: genetic inherited X linked traits

Type Defect Incidence Prognosis Diagnosis Manifestations Medical mgt

Hemophilia A Genetic defect Rare disease Twenty-five Diagnosed in Hemorrhage- severity Infusion of fresh
that results in Occurs I: 5000 percent of childhood. depends on the degree of frozen plasma
Factor VIII births Type A will factor deficiency
defect or A is 3x more develop Bleeding can be IV Factor VIII &
deficiency common than inhibitor spontaneous or from IX concentrates
Hemophilia B antibodies trauma Amicar
that diminish Bleeding occurs: Joints DDAVP
Almost all effectiveness (5%) GI tract; Mucus (Desmopressin)
Males female of treatment membrane; intra/ extra Plasmapheresis
carriers) and make cranial. Factor V!!a
them to Pain , Ankylosis Immunosuppressan
susceptible to Spontaneous hematomas t therapy.
Hemophilia B Accounts for blood can compress adjoining
(Christmas Factor IX defect 12-15% of infections nerves.
disease) or deficiency patients Types A are more likely
Fifty percent to be crippled from
of type B hemarthroses. They face
hemophiliacs the least hopeful
develop prognosis of all of the
inhibitor hemophilia types.
antibodies.
DIC **Not a disease . Treatment: treat
RISK but a sign of an underlying cause,
FACTORS: underlying correct tissue
Blood disorder. ischemia
transfusion Replace fluids and
Severity is electrolytes,
reaction variable; may be Maintain blood
Cancer,
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especially life-threatening. pressure

certain types Replace
coagulation factors
of leukemia Triggers may Use of heparin
include sepsis,
Inflammation trauma, shock
of the cancer, abrupto
pancreas placenta, toxins,
(pancreatitis) and allergic
reactions.
Infection in
Altered
the blood,
homeostasis
especially by mechanism
bacteria or causes massive
fungus clotting in
microcirculation
Liver disease . As clotting
Pregnancy factors are
consumed,
complications bleeding occurs.
Recent Symptoms are
surgery or related to tissue
anesthesia ischemia and
bleeding.
Severe tissue
Laboratory tests.
injury
All coagulation
studies,
Large
electrolytes,
hemangioma CBC
vessel that is
not formed
properly)

****** DIC: DISSEMINATED INTRAVASCULAR COAGULATION Not a disease but a sign of underlying disorder****
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