HAEMATOLOGY
3RD YEAR MBBS
SPIRAL II
STUDY GUIDE 2014
SHIFA COLLEGE OF MEDICINE
CONTENTS
Introduction ………………………………………………………… 3
Themes of the module ……………………………………… 6
Table of Specification ……………………………………… 7
CASES
Theme 1: Pallor ………………………………………………… 10
Theme 2: Infection/Leukemia ………………………. 29
Theme 3: Bleed/Clot ………………………………………… 57
Theme 4: Transfusion Medicine …………………… 66
Resources for learning ………………………………………… 75
People to contact ………………………………………………… 77
Glossary ………………………………………………………………… 78
INTRODUCTION:
On behalf of my team, I welcome you on board. This four week module of HAEMATOLOGY& TRANSFUSION MEDICINE is the second level of learning basics of CLINICALHAEMATOLOGY.(Ist level, we have completed in year I).The last & the third part of Haematology undergrad curriculumwill be taught during your fourth year clerkship. This last part(4th year) is vertically integrated to the third year part, along all themes but with more emphasis on haematological malignancy, where you would apply this basic knowledge in management including diagnosis and treatment of blood disorders.
The module works around four basic themes of clinical Haematology( you are already familiar with!!) PALLOR, HAEMATOLOGICAL FEVER, BLEED/CLOT & TRANSFUSION MEDICINE.
First Theme Pallor(6 days) would address all causes of clinical anemia, getting into details of the parasitic causes, structural defects of red cells and extracorpuscular causes of anemia.
Second Theme of Haematological Fever(5 days) in the module deals with, infectious causes of fever and blood cell variations in detail (your subject of assessment this year: microbiology).It also include an introduction to the malignant causes of febrile illness in order to develop a good differential ability in students for fever with blood cell dyscrasias.
The Third Part(4 days) of the module would deal with the concept of clinical bleed & basic clotting problems. The concept of DVT you have already learnt in CVS module, so here we would learn details of common clotting problems & basics of “THROMBOPHILIA”
Fourth Section of the module is related to basic knowledge of Transfusion Medicine(4 days).It was especially included in undergrad curriculum, considering the clinical problems faced by young doctors working independently/in a setup where still some accountability exists in medical care!.
Availability & use of alternate blood groups & picking up immediate transfusion reactions are two main objectives addressed in this part of the module.
We will have our feedback in the middle and at the end of the module.My team and I, are all the time available to you (your study guide has our e-mails!!) during and even after the module for any assistance in your learning and we expect you to behave and show maximum interest throughout the module. I hope and pray that you would make best use of the learning aids available to you.
Happy learning & good luck!!
THEMES: HAEMATOLOGY
PALLOR
FEVER (INFECTION, LEUKEMIA)
BLEED/CLOT
TRANSFUSION MEDICINE (TRANSFUSION REACTIONS)
TABLE OF SPECIFICATION
ASSESSMENT
MCQ 70%
SAQ 30%
IPE 30%
DAYS ALLOCATION 19 DAYS
THEMES
• PALLOR 6 DAYS
• HAEMATOLOGICAL FEVER 5 DAYS
• BLEED & CLOT 4 DAYS
• TRANSFUSION MEDICINE 4 DAYS
TABLE OF SPECIFICATION
Pallor 25%
Haematological Fever 45%
Bleed /Clot 15%
Transfusion Medicine 15%
OBJECTIVES
PALLOR
Formulate differential diagnosis of anemia on the basis of history and physical examination
Order appropriate & specific laboratory tests (after Interpreting peripheral film& CBC results) for defining the etiology of anemia
Manage different types of anemia in emergency
Describe iron, folate & Vit B12, preparations (hematinics) with regard to
Clinical use, Cost effectiveness, MOA & drug interactions
Advise parents/patients appropriately for long term management of inherited types of anemias & Counsel about genetic inheritance
Haematological Fever
Infection/leukemia
Describe haematological aspects of infectious fever(Malaria, Visceral Leishmaniasis, Typhoid fever, Viral haemorrhagic fever, Dengue Fever with regard to
pathogenesis,
clinical features
laboratory diagnosis
prevention
Counsel about the prevention of Malaria & Leishmania
Describe haematological aspects of infectious fever(Malaria,Visceral Leishmaniasis, Typhoid fever, Viral haemorrhagic fever,Dengue Fever with regard to
pathogenesis,
clinical features
laboratory diagnosis
Prevention
Counsel about the prevention of Malaria, & Leishmania
•Suspect hematological malignancy on the basis of history and physical examination
For fourth year level(vertical integration)
Describe various types of leukemias/lymphoma
•Order and interpret appropriate tests for precise diagnosis (morphology, immunophenotyping, cytochemistry & cytogenetics)
•Order and interpret appropriate staging work-up (lymphoma, MPD & MDS)
•Describe the chemotherapeutic agents and their disfiguring side effects
BLEED/CLOT
Counsel patients/ parents about the mode of inheritance & advise long term managementof Haemophilia
•Diagnose & Manage a case of snake bite
•Diagnose, counsel & manage a case of thrombophilia
•Prescribe basic drugs for a bleeding and thrombophilic patient
Transfusion medicine
Describe clinical application of blood group compatibility
State Judicious & appropriate use of blood components & products
State standardized screening tests for blood
State the process of safe storage and transport of blood and blood products
Describe hazards of blood transfusion
Appreciate the clinical presentation of immediate transfusion reactions (hemolytic, allergic & anaphylactic types)
Show ethical & Social awareness about blood donation (voluntary/ paid, donor deferral, cost of products)
CASES
THEME: PALLOR
IRON DEFICIENCY ANEMIA
MEGALOBLASTIC ANEMIA
THALASSEMIA MAJOR
SICKLE CELL ANEMIA.
APLASTIC ANEMIA
THEME: INFECTION/LEUKEMIA
MALARIA
VISCERAL LEISHMANIASIS
TYPHOID& PARATYPHOID FEVER
DENGUE HAEMORRHAGIC FEVER
HUMAN IMMUNODEFICIENCY VIRAL INFECTION
THEME:
IDIOPATHIC THROMBOCYTOPENIC PURPURA (ITP)
HAEMOPHILIA A
HAEMOPHILIA B
VON WILLEBRAND DISEASE
TTP/HUS
DIC
THEME:
BLOOD GROUPING & CROSS MATCH
VOLUNTARY & SAFE TRANSFUSION
ACUTE ALLERGIC TRANSFUSION REACTION
ACUTE HEMOLYTIC TRANSFUSION REACTION
THEME : PALLOR
(CASE 1)
IRON DEFICIENCY ANEMIA
PRESENTING COMPLAINTS
A 15 month old boy has presented in Pediatric OPD with C/O lethargy and pallor. He is less playful and inactive for few months.
HISTORY OF PRESENT COMPLAINT
The mother brings the toddler, as a visiting relative, who has not seen the child for 5 months told his mother that the boy appears very pale. He used to be an active & playful child but now appears lethargic. There is no change in his sleeping habits.
Upon enquiry, his mother told that he never had blood on his diapers and no black or tarry stools. He is a picky eater, taking small amounts of chicken, and some vegetables, but loves milk and drinks six to eight bottles of whole milk per day.
PAST MEDICAL HISTORY
Nothing significant
MEDICATIONS: No knownallergy to any medicine
BIRTH HISTORY; normal vaginal delivery, born in a district hospital, Fully vaccinated
FAMILY HISTORY
Elder and only sibling(boy) had the same complaints in his age of being pale & lethargic
GENERAL PHYSICAL EXAMINATION:
VITAL SIGNS: TEMP 37.50C, BP 90/52, PULSE 145/min,
RR 16/min
Pale appearing, inactive toddler, holding a bottle, tearing and eating paper
Eyes: No scleral icterus. Pale conjunctiva.
CENTRAL NERVOUS SYSTEM
Higher Mental Function: intact.
Motor and sensory system: intact.
No cranial nerve deficit.
CARDIOVASCULAR SYSTEM
Mild tachycardia , grade II systolic ejection murmur heard best over the upper left sternal border.
RESPIRATORY SYSTEM
Normal vesicular breathing
GASTROINTESTINAL TRACT
Abdomen: No hepatosplenomegaly.
Rectal examination: Dark brown, soft stool, negative for occult blood.
INVESTIGATIONS:
Laboratory investigations:
COMPLETE BLOOD COUNT:
WBC: 6,100/ul, Hgb: 6.2 g/dl, Hct: 19.8%, Plt : 589,000/ul,
MCV: 64 fl, RDW 17%.
RETICULOCYTE COUNT is 1.0%.
PERIPHERAL FILM: microcytosis, hypochromia, mild anisocytosis and polychromasia. There is no basophilic stippling.
Radiology : NOT REQUIRED
Special investigations:
Serum Iron: 15ug/dl ( Male: 59-158 ug/dl,
female: 37-145 ug/dl)
TIBC: 450ug/dl ( 228-428 ug/dl)
Serum ferritin: 4.25ng/ml (Male: 22.0-275.0ng/ml,
female : 5.0-204.0ng/ml)
Transferrin receptor study : Normal
Critical questions/ study questions
Q.1 What measures can you take to prevent parasitic infestation in Pakistani rural children?
Q.2 How can you ensure appropriate Iron intake in low socioeconomic groups in your society?
(CASE: 2)
MEGALOBLASTIC ANEMIA
PRESENTING COMPLAINTS
A 52 year old female presents with pallor, tingling in hands and feet and decrease sensation in feet.
HISTORY OF PRESENT COMPLAINT
Presents with pallor and tingling sensation in both feet. She is a strict fad dieter.The complaints were progressive in onset and were not accompanied by any fever or h/o seizures or vertigo . No Hx of trauma and any transfusion. No H x of unconsciousness or vomiting
PAST MEDICAL HISTORY
Nothing significant
Medications,allergy; nil
Hospitalization/surgery; nil
Birth history; normal vaginal birth
FAMILY HISTORY
Two brothers, one sister
Cardiac problem runs in the family
Sexual/ social hx; Nothing significant
GENERAL PHYSICAL EXAMINATION;
VITAL SIGNS: TEMP 38C, BP 120/80mm, PULSE 100/min, RR 18/min
Eyes: No scleral icterus. Pale conjunctiva.
Mouth: No Dental caries. Satisfactory oral hygiene
CENTRAL NERVOUS SYSTEM
Higher Mental Function: intact.
No cranial nerves deficit.
Motor system: Gait with sensory ataxia.
Knee reflex Normal
Ankle jerk Absent
Upgoing planters bilateral
Sensory exam: impaired position and vibration sense in both
lower limbs.
CARDIOVASCULAR SYSTEM
Normal heart sounds.
RESPIRATORY SYSTEM
Normal vesicular breathing
GASTROINTESTINAL TRACT
Abdomen: No hepatosplenomegaly.
RectalExamination: Dark brown, soft stool, negative for occult blood.
INVESTIGATIONS;
Laboratory investigations:
COMPLETE BLOOD COUNT:
WBC 35,00/ul,100, Hgb 6.2 g/dl, Hct 27%, Plt 139000/ul
PERIPHERAL FILM: Pancytopenia, Hypersegmented neutrophils,
Radiology: Not Applicable
Special investigations:
Serum folate: 10.0ng/ml (3.0-17 ng/ml)
Red Cell folate: 617/32ng/ml (263-1028ng/ml)
Serum B12: 150.0pg/ml (243-894pg/ml)
Schilling test: Positive
Serum Bilirubin: Raised
LDH level : Raised
Intrinsic factor antibodies: Present
Critical questions/ study questions
Q.1 How can you preserve folates in diet?
Q.2 What are the common causes of megaloblastic anemia in Pakistan?
(CASE: 3)
THALASSEMIA MAJOR
PRESENTING COMPLAINTS
A 08 month old boy presents with lethargy, marked pallor, inactivity and abdominal distension.
HISTORY OF PRESENT COMPLAINT
08 month old infant presents with the marked pallor and growth failure. There is also Hx of change in facial appearance. Initially symptoms were less marked. But now they have progressed further.
PAST MEDICAL HISTORY
Nothing significant
Medications, allergy; nil
Hospitalization/surgery; nil
Birth history; normal vaginal birth
FAMILY HISTORY
H/O of death of sibling at the age of 15 months diagnosed as deficiency of blood
Sexual/ social hx; Not Applicable
GENERAL PHYSICAL EXAMINATION;
VITAL SIGNS: TEMP: 37.5C, BP 90/52, PULSE 140/min, RR 19/min
Pale appearing, inactive toddler,
Eyes: No scleral icterus. Markedly Pale Conjunctiva.
CENTRAL NERVOUS SYSTEM
Higher Mental Function: intact.
Motor and sensory system: intact.
No cranial nerves deficit.
CARDIOVASCULAR SYSTEM
Mild tachycardia as above, grade II/VI systolic ejection murmur heard best over the upper left sternal border.
RESPIRATORY SYSTEM
Normal vesicular breathing
GASTROINTESTINAL TRACT
Abdomen: Moderate Hepatosplenomegaly. No ascites, Normal Bowel sounds
RectalExamination : Dark brown, soft stool, negative for occult blood.
LABORATORY INVESTIGATIONS:
COMPLETE BLOOD COUNT:
Hb: 5gm/dl, TLC: 18,000/ul Platelet count of 150,000/ul.
RETICULOCYTE COUNT: 10%.
Peripheral Film: Marked poikilocytosis, anisocytosis, microcytosis, hypochromia, polychromasia target cells, many fragmented red cells, many NRBC.
Radiology : X-ray skull show crew cut appearance and maxillary prominence
Special investigations:
HPLC : HbF: 90%
HbA: 08%
HbA2:02%
Critical questions/ study questions
Q.1 What is the pathogenesis of Thalassemia?
Q.2 How is the disease prevalence related to social-cultural background of Pakistan?
Q.3 What can you do about poor children affected by the disease?
CASE: 4
SICKLE CELL ANEMIA.
PRESENTING COMPLAINTS:
A 10-year-old male child presents in ER with C/O severe pain "all over his body especially in his legs
HISTORY OF PRESENT COMPLAINT:
His mother brought him into the ED at 4 pm .She reported that the pain began early that morning and had "gotten worse." She reported that it was not relieved by his usual doses of ibuprofen. He was given with strong IM pain killer. He got only minimal pain relief after receiving the medication. He reported that the slight relief was short-lived, and he continued to complain of unbearable pain through the night.
PAST MEDICAL HISTORY:
His past history is significant with many such hospital admissions and h/o repeated chest infections and a non healing ulcer on his right ankle.
Medications, allergy; nil
Birth history; normal vaginal birth
FAMILY HISTORY
H/O of similar episodes of pain crisis and chest infection in two of the 5 siblings.
Sexual/ social hx; Not Applicable
GENERAL PHYSICAL EXAMINATION;
VITAL SIGNS: TEMP: 37.5C, BP: 110/70mm, PULSE: 140/min, RR 19/min
Pale appearing child in agony oriented in time, space and person having a chronic ulcer on right ankle.
Eyes: No scleral icterus. Markedly Pale Conjunctiva.
Mouth: Dental caries. Satisfactory Hygiene
CENTRAL NERVOUS SYSTEM
Higher Mental Function: intact.
Motor and sensory system: intact.
No cranial nerves deficit.
CARDIOVASCULAR SYSTEM
Moderate tachycardia , grade II/VI systolic murmur heard best over the upper left sternal border.
RESPIRATORY SYSTEM
Normal Vesicular Breathing
GASTROINTESTINAL TRACT
Abdomen: Moderate Hepatosplenomegaly. No ascites, Normal Bowel sounds
Rectal: Dark brown, soft stool, negative for occult blood.
LABORATORY INVESTIGATIONS:
COMPLETE BLOOD COUNT:
Hb: 5gm/dl, TLC: 12,000/ul Platelet count of 150,000/ul.
Reticulocyte Count: 12%.
Peripheral Film: Moderate poikilocytosis, anisocytosis,, hypochromia, polychromasia target cells, many fragemented and sickle red cells, many NRBC.
Radiology :
Special investigations:
Sickle Screening test: Positive
HPLC: HbS 70%
HbF: 13%
HbA: 17%
Critical questions/ study questions
Q.1 Why does sickling occur at time of stress/infection?
Q.2 What is the confirmatory test for establishing sickle trait?
CASE: 5
APLASTIC ANEMIA
PRESENTING COMPLAINT:
Bleeding from nose for past 2 hours
HISTORY OF PRESENTING COMPLAINT:
A previously healthy 3 year old girl presented to emergency department with the complaint of severe bleeding from nose for past 2 hours, which is poorly controlled by application of pressure. There is no other associated complaint.
PAST MEDICAL/DRUGS HISTORY:
Patient had otitis media 3 months ago that improved after treatment with chloramphenicol.
GENERAL PHYSICAL EXAMINATION:
Patient is well-oriented in time place and person.
All vital signs are within normal limits. However, examination is remarkable for marked pallor, and numerous petechiae and ecchymoses on her body.SYSTEMIC EXAMINATION:
CARDIOVASCULAR SYSTEM:
Apex beat localized in left fifth intercostals space in mid-clavicular line. No abnormal sounds heard on auscultation.
RESPIRATORY SYSTEM:
Normal vesicular breathing. No rhonchi / crepitations.
GASTROINTESTINAL SYSTEM:
No tenderness. No hepatosplenomegaly. Bowel sounds normal.
CENTRAL NERVOUS SYSTEM:
No sensory, motor or cognitive deficit.
Laboratory Investigations
Complete Blood Count:
Hemoglobin 4.5 g/dL
Hematocrit 15%
MCV 80 fl
Total Leukocyte Count 2000/mm3
Reticulocyte Count 0.2%
Platelet Count 12,000/mm3
Bone Marrow Biopsy
Shows a hypocellular marrow, replaced by large amounts of adipose tissue.
Critical questions/ study questions
Q.1 What are the common causes of Aplastic anemia in Pakistan?
Q.2 What is the curative treatment for this disease & how can every aplastic patient one get an access to this
Curative modality
Q.3 What are the side effects/hazards of Bone Marrow transplant?
LEARNING OBJECTIVES
At the end of this theme of pallor, students should be able to
1. formulate differential diagnosis of anemia on the basis of history and physical examination
2. order appropriate & specific laboratory tests after Interpreting peripheral film& CBC results, for defining the etiology of anemia
3. describe iron, folate & Vit B12, preparations (hematinics) regarding clinical use,cost effectiveness,MOA & drug interactions
4. perform Complete Blood Counts(K,S)
5. manage different types of anemia in emergency & in OPD(K,S)
6. diagnose and manage acute and chronic toxicity of iron(K,S)
7. advise parents/patients appropriately for long term management of inherited types of anemias & Counsel about their genetic inheritance (Community Health Advocate)
8. counsel & educate patients of deficiency anemia (diet in anemia)
9. communicates professionally with patients, parents/ caretakers and with paramedical staff regarding treatment and care of the patient,especially suffering from genetic disorders.
THEME: 2
INFECTION (HAEMATOLOGICAL FEVER)
CASE 6
MALARIA I
PRESENTING COMPLAINT
Fever- 1week
Headache- 1 week
Myalgia- 1 week
HISTORY OF PRESENT COMPLAINT
Patient was in usual state of health one week back when he started having complaints of high grade intermittent fever associated with rigor and chills, headache and bodyaches.
PAST MEDICAL HISTORY
Nothing significant
Medications, allergy: nil
Hospitalization/surgery: nil
Birth history: normal vaginal birth
FAMILY HISTORY
Nothing significant
Sexual/ social hx: Nothing significant
TRAVELING HISTORY
History of travelling from Nigeria 2 weeks back
GENERAL PHYSICAL EXAMINATION;
VITAL SIGNS: TEMP 1020F, BP 120/75mmHg, PULSE 90/min, RR 16/min
Patient febrile, appears lethargic. .
Eyes: No scleral icterus; Conjunctiva pale
Mouth: NAD
CENTRAL NERVOUS SYSTEM
Higher Mental Function: intact.
Motor and sensory system: intact.
No cranial nerves deficit.
CARDIOVASCULAR SYSTEM
Normal heart sounds.
RESPIRATORY SYSTEM
Normal vesicular breathing
GASTROINTESTINAL TRACT
INVESTIGATIONS:
Laboratory investigations:-
COMPLETE BLOOD COUNT:
WBC: 3,000/ul; Lymphocytes: 44%; Hb: 9.8 g/dl; Platelets: 62,000
ESR: mm/hr
LIVER FUNCTION TESTS: Within normal limits.
PROTHROMBIN TIME: 19 seconds
TOTAL SERUM PROTEINS: 93 g/L
SERUM UREA, CREATININE AND ELECTROLYTES:
SERUM LDH: 505 IU/L
Radiology:
ULTRASOUND ABDOMEN: Hepatosplenomegaly
Special investigations:
BLOOD CULTURES: Negative
WIDAL TEST: Negative
THICK AND THIN BLOOD FILMS FOR MALARIA: