Assessment and management

of Patients with Hematologic

Disorders

Joji B. Castellano, R.N.

SWU – College of Nursing
Lymphoid malignancies
Relative Frequency of Lymphoid Malignancies
Agnogenic Myeloid Metaplasia Lymphoma

Lymphomas
 These are neoplasms of the lymphoid tissues
(e.g. lymphocytes and histiocytes)
 Despite the name, all of these disorders are
malignant
 Hodgkin’s Lymphomas are distinguished from
Non-Hodgkin’s Lymphoma by the presence of
Reed-Sternberg Giant Cells
 Reed- Sternberg Cells
 These are binucleate cells that usually
display prominent inclusion-like nucleoli
surrounded by lymphocytes
 The Reed-Sternberg Cell is a necessary but
insufficient pathologic finding for the
diagnosis of Hodgkin’s Disease
Mixed cellularity Hodgkin's
disease. A Reed-
Sternberg cell is present
near the center of the field;
a large cell with a bilobed
nucleus and prominent
nucleoli giving an "owl's
eyes" appearance. The
majority of the cells are
normal lymphocytes,
neutrophils, and
eosinophils that form a
pleiomorphic cellular
infiltrate
 Non-Hodgkin’s
Risk Factors EBV; 7-11 years old
Histology Varies
Disribution Noncontiguous lymph node
spread
Variants Many separated into low,
intermediate & high grade
History Painless adenopathy
PE Systemic adenopathy;
hepatosplenomegaly
Differential Hogkin’s; cat-scratch disease,
HIV, mononucleosis; sarcoid
Evaluation Biopsy for diagnosis; CXR, whole
body CT; BM biopsy & LP
Staging Based on # of nodes; whether
disease crosses diaphragm
Treatment Radiation and Chemotherapy
Hodgkin’s
EBV; bimodal age distribution: 15-45
& >60 years old
Reed-Sternberg Cells
Contiguous spread
Nodular sclerosis; mixed cellularity, lymph-
ocyte predominant; lymphocyte depleted
Painless adenopathy; pruritus
Regional adenopathy
hepatosplenomegaly
Non-Hodgkin’s;HIV; sarcoid; drug
reaction; lymphadenitis
Biopsy for diagnosis; then CXR; BM
biopsy & LP
Same
Radiation for local dse; radiation for
advanced/widespread disease
 Chemotherapeutic Agents
Non-Hodgkin’s Hodgkin’s Lymphoma
C Cytoxan A Adriamycin M Mechlorethamine

H Adriamycin B Bleomycin O Oncovin

O Oncovin V Oncovin P Prcarbazine
(Vincristin) (Vincristin
P Prednisone D Dacrbazine P Prednisone
 Multiple myeloma
 A malignancy of plasma cells resulting from the
expansion of cells within bone marrow, often with
unbalanced, excessive production of immunoglobulin
heavy/light chain
 Radiation, chemical exposure and monoclonal
gammopathy of undetermined significance (MGUS) are
risk factors
 Patients >50 years old are most commonly affected
 Blacks are affected more frequently than whites
 History & PE
 Symptoms include back pain, hypercalcemic
symptoms (“stones, bones, abdominal moans &
psychiatric overtones”); pathologic fractures, fatigue
& frequent infections

 Exam may reveal pallor, fever, bone tetnderness, bone

deformities & lethargy
 Evaluation
 Serum and Urine Electrophoresis; Bence-Jones
proteinuria, CBC, Peripheral Blood Smear;
Electrolytes; Full-Body Skeletal Survey (X-rays
showing Punched-Out Osteolytic lesions of the skull
and long bones)
 Bone scans and Bone Marrow Biopsy (Plasma Cell
infiltrate)
 Staging is based on the degree of anemia, elevated
calcium, xray findings and renal dysfunction
 Treatment
 Multiagent Chemotherapy, including alkylating
agents, melphalan and prednisone
 Consider Stem Cell Bone Marrow Transplantation
 Fractures may require intramedullary Fixation
 Disease usually recurs and carries a poor prognosis
 Complications include anemia, infection, neurologic
disease and renal failure
Bleeding Disorders
Idiopathic Thrombocytopenic Purpura (ITP)

 An autoimmune platelet disorder that includes pediatric

and adult forms
 Females are affected twice as often as males
 Individuals >50 years of age are at greater risk
 Evan’s Syndrome is ITP with Autoimmune Hemolytic
anemia
 ITP may be associated with Hodgkin’s & Non-Hodgkin’s
Lymphoma, CLL, HIV,SLE and RA
History and PE

 Unlike TTP, patients are afebrile and splenomegaly

is absent

 Exams reveal petechiae, purpura, ecchymoses,

mucosal bleeding (epistaxis, oral bleeding,
menorrhagia), and oral hemorrhagic bullae
Evaluation
 CBC shows very low platelets, possibly with anemia
 A normal PT and PTT, prolonged bleeding time, a
normal DIC panel, and a positive platelet-associated
IgG test may be present
 A peripheral blood smear will show megathrombocytes
without schistocytes
 Bone Marrow aspiration will show either normal or
increased megakaryocytes
Treatment
 This condition will resolve spontaneously
in most pediatric patients
 Adults are treated with corticosteroids
 Platelets should be given for severe bleeding
 IVIG & anti-Rho (D) (for Rh-positive patients) are given
for life-threatening hemorrhage
 Splenectomy should provide effective treatment for
2/3rds of patients who are refractory to medical
treatment
 Thrombotic Thrombocytopenic Purpura (TTP)

Causes HIV Infections; Pregnancy; OCP use

Individuals <50 years of age

Manifestati Pentad of fever, anemia, spleniomegaly, TCP,

ons ARF & neurologic abnormalities

Laboratory Anemia, TCP, schistocytes, ↑ B1, ↑ LDH, (-)

Findings Coomb’s Test, hyaline thrombi in small vessels
without inflammatory changes
Treatment Large Volume Plasmapheresis, Corticosteroids,
ASA; Splenectomy
Coagulation Disorder: Hemophilia

 These are X-linked recessive coagulopathies due to:

 Factor VIII deficiency: Hemophilia A
 Factor IX Deficiency : hemophilia B

The classic case of hemophilia is the boy (X-linked) from the

Imperial Russian family (recessive) who presents with
hemarthroses following minimal or no trauma
 History and PE
 Patients present with soft tissue hemorrhage,
including, hemarthroses, intramuscular bleeding, GI
bleeding and excessive bleeding from mild trauma
and surgical/dental procedure
Evaluation
PT Normal
PTT Increased
Bleeding Normal
Time
Platelets Normal
Ristocetin Normal
Cofactor
Assay
 Von Willebrand’s Disease
 An autosomal-dominant condition resulting in deficient
or defective von Willebrand factor (vWF)
 It is the most common inherited bleeding disorder
 It is characterized by decreased platelet adhesion with
a concurrent deficiency of factor VIII
 Patients present with easy bruisability, mucosal bleeding
and post-incisional bleeding
 The decreased platelet function is usually not sufficiently
severe to produce petechiae
 Symptoms worsen with ASA use
 Suspect this diagnosis in a woman with heavy
menstrual bleeding, especially if she has a family history
of bleeding disorder
 Treatment includes Desmopressin for mild disease,
OCP for menorrhagia, and FFP or Cryoprecipitate for
major bleeding
 ASA and ASA-containing products should be avoided
 Transfusions should be minimized to decrease the risk
of transmitted viral infection
Disseminated Intravascular Coagulopathy (DIC)

Causes Sepsis; transfusion reactions, neoplasia, trauma

obstetric complications (e.g amniotic embolus,
septic abortion)
Manifestati Bleeding from venipuncture sites, epistaxis,
ons hematemesis, digital gangrene, hypotension

Laboratory Anemia, TCP, ↑PT/PTT, ↑Bleeding Time, ↑ d-

Findings dimer, ↑fibrin split products, ↓fibrinogen

Treatment Treat the underlying condition; transfuse with

platelet and cryoprecipitate
 Hypercoagulable States

 A group of condition that predispose patients to

thromboembolic disease, including DVTs,
Pulmonary emboli, and CVAs
 Patients often present with venous thrombosis or
recurrent thromboembolism in adolescence or early
adult life
 Factor V Leiden is the most common inherited
hypercoagulable disorder
 CAUSES OF HYPERCOAGULABLE SATES
INHERITED ACQUIRED
Factor V Leiden Mutation Prolonged bed rest
Protein C or S deficiency MI
Antithrombin III Deficiency Tissue damage
Homocystenemia DIC
Fibrinolysis Defects Hyperlipidemia
Vasculitis
Multiple Myeloma
Lupus Anti-coagulant
Nephrotic Syndrome
Smoking
Cancer
OCP/Warfarin
Pregnancy
 LONG TERM
COMPLICATIONS OF
DIABETES MELLITUS
COMPLICATIONS OF DM
 MACROVASCULAR COMPLICATIONS

 Diabetics are prone to have high bad cholesterol.

 When cholesterol is too high, the walls of large blood
vessels become narrowed, even clogged.
 This problem is called atherosclerosis.
 MACROVASCULAR COMPLICATIONS

 Narrowed and clogged blood vessels decrease blood flow to

all parts of your body especially the heart and the brain.
 This can lead to heart attack and stroke.
 MICROVASCULAR COMPLICATIONS

 Longstanding high blood sugar also damages the

small blood vessels supplying the eyes, kidneys and nerves
of the feet.

 Erectile difficulty and impotence may happen due to

damage to nerves going to the sexual organ.
 Diabetic retinopathy

BLINDNESS Vitreous Hemorrhage

Proliferative

Normal retina Preproliferative

Diabetic Neuropathy
Autonomic
Neuropathy

Constipation

Peripheral
Neuropathy Erectile difficulty
Diabetic Foot
Diabetic Nephropathy

Protein in
urine
 POORLY-CONTROLLED
DIABETICS are …

 2-4x more prone to HEART DISEASE

 25x more prone to BLINDNESS
 90x more prone to NEUROPATHY
 5x more prone to GANGRENE
 17x more prone to KIDNEY DISEASE
HOW DO I PREVENT
COMPLICATIONS?
Guarding Your
Heart
 Guarding the Heart
 Keep bp under 130/80 mmHg
 Keep blood sugar controlled
 Keep cholesterol controlled
 LDL (bad cholesterol) < 100mg/dL

 HDL (good cholesterol) > 40 (men) > 50

(women)
 Triglyceride < 150mg/dL


Exercise. Lose weight if needed.
Quit smoking
Take an aspirin once a day.

Guarding Your
Kidneys
Guarding the Kidneys
 Keep bp under 130/80mmHg
 Keep blood sugar well-controlled
 Avoid dehydration. If there's vomiting
or diarrhea, consult your doctor.
 Do not self-medicate especially with
painkillers.
 If you have proteinuria, see a dietitian

for individualized meal planning.

Guarding the Kidneys
 See a doctor for bladder or kidney infections.
 You may have an infection if you have:
 pain or burning when you urinate
 frequent urge to go to the bathroom
 urine looks cloudy or reddish
 fever or chilliness
 back pain or on the side below the ribs
Caring for Your
 PROPER EYE
CARE
Good blood sugar
control.

Visit your ophthalmologist

at least once a year.
Medications
OAD
Insulin