CASE PRESENTATION

ACUTE LYMPHOID LEUKEMIA

(AIRWAY MANAGEMENT)

By: Dr. Govind K. Goyal

Moderator: Dr. Ramesh C. Sunar
 HISTORY
Name- Raghavendra s/o Deenanath
Age- 15 Yrs
Sex- Male
Residence- Gangapur (Bhilwara)
Date of admission- 2/12/2011

Chief complain- Fever since 2 months

Pain in throat since 10 days
 HISTORY OF PRESENT ILLNESS

 Pt. has fever for 2 months. It is persistent type, dull in

nature, low grade fever. Slightly decreased by taking
Tab.Paracetamole.
 Pt.has started throat pain for 10 days with difficulty
in respiration.
 PAST HISTORY

 No H/O same disease in past

 No H/O Previous surgery
 No H/O Chronic illness
 PERSONAL HISTORY

 Birth history- G2P1

 Delivered by CS
 At birth BB – 2.2 KG
 AAA Pt. is pure vegetarian
 No motor developmental milestone delay
 No H/O any Drug allergy
 Fully vaccinated as schedule
 No H/O diarrhoea, vomitting
 EXAMINATION
 Awake, conscious, oriented, restless & anxious
 Pallor ++
 spleenomegaly
 cervical Lymphadenopathy
 Icterus – , clubbing – , Cyanosis –

PR - 120/min Regular, Normal volume in character

 All peripheral pulses palpable, No radio-femoral delay

 BP – 100/70 mm Hg

 Temperature febrile on touch

 Respiration is laboured and rate 28 /min
 CONT…
 On auscultation, air entry is decreased in bilateral lung
fields;
 heart sounds is normal.
 Petechiae spots, splinter hemorrhages and bilateral
conjunctival hemorrhage present.
 There is a large swelling all around the neck and on
face. Mouth opening is one and a half fingers.
 Tongue is swollen and oral mucosa is edematous with
bluish discoloration suggestive of hematoma.
 Modified Mallampatti Grade is III.
 X-ray soft tissue neck, in lateral view showed swollen
lips, face, tongue, epiglottis and soft tissue of neck.
 The shadow of air passage in neck is fairly adequate
 INVESTIGATION
 Hemoglobin: 5.5 gm/ dl
 Total leucocyte count: 3000 /cmm
 differential leucocyte count: polymorphs 20, lymphocytes
55,eosinophill 3, monocytes 2; blast cells: 20%;
reticulocytes 0.2%;
 Platelet count 30,000 /cmm
 Bone marrow examination showed predominantly blast
cells.
 Blood culture was sterile
 Widal test negative
 RFT/ LFT Within normal range
 ECG- Bradycardia
PROVISIONAL DIAGNOSIS

A provisional diagnosis of Acute

Lymphoid Leukemia (L2 type) is
made
 BLOOD
 Blood is a specialized bodily fluid consisting of plasma,
blood cells, and platelets that is circulated by the heart
through the vascular system, that delivers necessary
substances such as nutrients and oxygen to the cells and
transports metabolic waste products away from those
same cells.
 Blood accounts for 8% of the human body weight, with
an average density of approximately 1060 kg/m 3, very
close to pure water's density of 1000 kg/m3.
 The average adult has a blood volume of roughly 5 liters
(1.3 gal).
 CONSTITUTES
BLOOD
BLOOD CELLS (45%) PLASMA (55%)

1. RBC 1. Water(92%)
2. WBC 2.Proteins(Albumin)
3. Platelets 3.Ig
4.Blood Clotting factors
5. Lipoproteins
6.Electrolytes(Na&K)
7.Dissolved Nutrients
8.Bodily waste product
 BLOOD CLOTTING FACTORS
I. Fibrinogen
II. Prothrombin
III. Tissues plasminogen factor,Thromboplastin
IV. Calcium
V. Proaccelerin , labile factor
VI. Not named
VII. Proconvertin, Stable factor
VIII. Antihemophilic factor A
IX. Antihemophilic factor B Or Christmas factor
X. Stuart-Prover factor
XI. Plasma thromboplastin antecedent
XII. Hageman factor, Glass contact factor
CONT…

 The serum refers to plasma from which the

clotting proteins have been removed. Most of
the proteins remaining are albumin and Ig.

(Serum = Plasma – Clotting factors)

 Normal Blood pH is a within the narrow range of

7.35 to 7.45, making it slightly alkaline.
 RBC(ERYTHROCYTE)
 By volume, the red blood corpuscles constitute about 45% of
whole blood
 It is a circular, biconcave cell without a nucleus
 Diameter about 7.5μm
 Thickness at the periphery is about 2 μm and at the centre it
is 1 μm
 95% of the dry weight of the RBC is due to Hb.
 Hb is interwoven in the stroma of RBC so if a RBC is cut,
the Hb is not extruted
 Normal range 4.7 to 6.1 million/cmm(male), 4.2 to 5.4
million/cmm (female)
 Normal survival time is 120 days
 WBC(LEUCOCYTES)

 By volume the WBC constitute about 0.7% of whole

blood
 Normal range 4000-11000 /cmm of blood
 On the staining property by one of the Romanowski’s stain
(Leishman’sstain) they devided in to two types

Granulocytes
Non granulocytes

Neutrophil (60-70%) Lymphocytes (25-30%)

Eosinophil (1-4%) Monocytes (2-8%)
 NEUTROPHILS
 Diameter 10-12 μm
 Mature neutrophil have several lobes 2,3 up to 7
 Cytoplasmic granules are amphophilic, take both acidic and
basic stains
 Granules are two types

Primary/Azurophilic/Lysosomal Secondary/Specific

Proteolytic & Amylolytic granules Lactoferrin

MPO enzymes granules Alkaline phosphates
Lysosomal enzymes granules Vit.B12 binding proteins
 EOSINOPHILS
 Diameter 10-15 μm
 Usually bilobed
 Cytoplasmic granules are very coarse and contain alkaline
materials

Eosinophilic peroxidase enzymes

Major basic protines (50%)
Eosinophilic cationic protines
 BASOPHILS
 Diameter 8-10 μm
 Nucleus is irregular and often ‘S’ shaped
 Cytoplasmic granules are coarse and contain

Histamine
Heparin
Acid peptides
Acid hydrolases
Neutral proteolytic enzymes
 LYMPHOCYTES
 They are devided in to following types
Size Cell type

Small Large (10%) T-cells B-cells

8-10 μm 10-12 μm
( wholly consist ( cytoplasm abundant )
of nucleus,cytoplasm
occupying a narrow
rim round the nucleus)
 MONOCYTES
 Largest cell of WBC
 Diameter 12-20 μm
 Nucleus are horse shoe shaped appearance
 PLATELETS(THROMBOCYTES)

 Normal counts are about 1.5-4 lacs/cmm

 Diameter between 2-4 μm
 In the circulating blood they are in inactive state and have a
disc like structure
 When there is injury they become activate and spherical in
structure
 Critical count- if the count is below 40,000/cmm then
haemorrhagic manifestations started
 FUCTION
Blood performs many important functions within the body
including:-
 Supply of oxygen to tissues (bound to hemoglobin, which is carried
in red cells)
 Supply of nutrients such as glucose, amino acids and fatty acids
(dissolved in the blood or bound to plasma proteins (e.g., blood
lipids))
 Removal of waste such as carbon dioxide, urea, and lactic acid
 Immunological functions, including circulation of white blood
cells, and detection of foreign material by antibodies
 Coagulation, which is one part of the body's self-repair mechanism
(blood clotting after an open wound in order to stop bleeding)
 Messenger functions, including the transport of hormones and the
signaling of tissue damage
 Regulation of body pH
 Regulation of core body temperature
HEMATOPOIESIS,

Erythropoiesis Myelopoiesis

RBC WBC Platelets

CONT…
Blood forming tissues

Myeloid Lymphoid ( lymph nodes,

( red bone spleen, thymus )

marrow)

RBC Lymphocytes
WBC
Platelets
 CONT…

 3w to 3m of IUL- Mesoderm of the yolk sac

 3m to 5m of IUL- Liver & spleen
 5m to onwards - RBM
 In an adult RBM found only in the cranial bone, ribs,
sternum, vertebre, pelvic bones & in the upper end of
the long bones
PATHOLOGY

1. General medical disorders

Disorders of volume Disorders of circulation

Injury Shock
Dehydration Atherosclerosis
Thrombosis
 CONT…

2. Hematological disorders
Anemia Disorders of cell proliferation Disorders of coagulation

Hemophilia A & B
Sickle cell anaemia Vwd
Herediatary spherocytosis DIC
Thalassemia Aplastic TTP
PNH Polycythemia ITP
Megaloblastic anaemia Myelophthisic HUS
Pernicious anaemia Leukemia Thrombasthenia
iron defficiency anaemia Leucocytosis
Lymphoma
Multiple myeloma Infectious disorders of blood
Thrombocytosis
Thrombocytopenia Viral

Bacterial
Parasitic
 LEUKEMIA
 Leukemia is a Clonal malignant neoplasm of the
hematopoietic stem cells characterized by the
proliferation of abnormal (leukemia)blast cells and impaired
production of normal blood cells.
 It is classified on the basis of the cell type involved and
the state of maturity of the leukemic cells in to 2 types

Acute Chronic
(+ of immature cells) (well differentiated cells)

AML ALL CML CLL

 ACUTE LYMPHOBLASTIC LEUKEMIA

Immunologic % of Cases FAB Subtype Cytogenetic

Subtype Abnormalities

Pre-B cell ALL 75 L1,L2 t(9;22), t(4;11),

t(1;19)

T- cell ALL 20 L1,L2 14q11 or 7q34

B- cell ALL 5 L3 t(8;14), t(8;22),

t(2;8)
ALL –L1
ALL –L2
ALL –L3
 CONT…
 Acute lymphoid leukemias (ALLs) are
predominantly cancers of children and young
adults .
 Peak incidence approximately four years age.
 More frequent in boys than in girls.
 Exposure to high-energy radiation in early
childhood increases the risk of developing T cell
ALL.
 The cells are heterogeneous in size, have round
or convoluted nuclei, high nuclear/cytoplasmic
ratio, and absence of cytoplasmic granules.
 CONT…
 The leucocyte count may vary from 1000/cmm to 5
lacs/cmm.
 The appearance of blast cells in blood film is
usually diagnostic.
 Diagnosis of ALL is confirmed by demonstration of
20% lymphoblasts in the bone marrow.
 There is associated anaemia and
thrombocytopenia.
 The coagulopathies are usually mediated by
thrombin activation rather than primary fibrinolysis
and may lead to both hemorrhagic and thrombotic
complications.
 CONT…
 Pulmonary complications secondary to lymphoblastic
leukemia are pneumonia, pulmonary leukostasis,
malignant pleural effusion and / or pulmonary
infiltration and upper airway obstruction.
 The cause of upper airway obstruction can be
epiglottitis, enlarged lymph nodes, laryngeal mass
comprised of leukemic cells.
 In a lymphoid malignancy presence or combination of
nasopharyngeal obstruction, laryngeal displacement,
malignant infiltration of tonsils and pharynx and
potential tracheal compression put such a patient at
extreme risk for airway complications.
 COMPLICATIONS ARISING FROM
THE DISEASE
 Decreased resistance to infection
 Hyperviscosity syndrome— drowsiness,
coma,
focal neurological defects
 CNS involvement
 Anemia
 thrombocytopenia
 bleeding tendency
 DIC
 PROGNOSIS

Parameters Good poor

 WBC low High(>50x10 9 /l)

 Gender Girls Boys
 Age Child Adult or infant.
 Immunophenotype C-ALL B-ALL
 Cytogenetic Normal, Ph+,11q23
hyperdiploid rearrangements
 MANAGEMENT
1. General Management-:
 The increased white cell mass may be reduced by
leukophoresis.
 Frequent blood transfusions to maintain Hb levels at >7
g/dL.
 Platelet transfusions are required if counts remain <10 to
20,000 or if <50,000 and remaining symptomatic or
undergoing an invasive procedure.
 Give FFP and other blood products as needed.
 Neutropenia management, including protective isolation,
appropriate antibiotic therapy, ± granulocyte-colony
stimulating factor.
 GVHD is managed by supportive treatment and parenteral
nutrition. PGE1 and immunosuppression may be helpful.
 Psychological support for both patient and family is vital.
 MANAGEMENT CONT…

2. Medical therapy-:
 Therapy consists of a three-phase treatment
induction, CNS prophylaxis, and maintenance
 Induction therapy with prednisone, vincristine, L-
aspara-ginase, and daunorubicin
 Maintenance therapy with 6-mercaptopurine,
methotrexate, cyclophosphamide, and prednisone
is given in a cyclical fashion for 2 or more years.
 CONT…
Complications arising from the therapy-
 Tumor lysis syndrome—Hyperkalemia, hyperuricemia, and
acute renal failure may follow rapid destruction of a large
white cell mass.
 Neutropenia and immune compromise with an increased
risk of infection
 Anemia
 Thrombocytopenia leading to spontaneous bleeding, usually
from intravascular catheter sites, skin, lung, gut, and brain
 Lung fibrosis (e.g., after radiotherapy, bleomycin)
 Myocardial failure (e.g., after mitoxantrone)
 Graft versus host disease (GVHD)—Features include
mucositis, hepatitis, jaundice, diarrhoea, abdominal pain,
rash, and pneumonitis
 AIRWAY MANAGEMENT
 Airway obstruction is the primary mechanical
emergency of the respiratory system in patients with
malignancy.
 Obstruction can occur at the level of larynx, trachea or
bronchi.
 Symptomatic relief is the main objective for patients
with airway obstruction.
 Patient should be allowed to assume position of
maximum comfort.
 If pt. unable to maintaine spontaneous respiration then
intubation done.
CONT…
 On operation table, monitoring for heart rate,
ECG, non - invasive blood pressure(NIBP) and
pulse oximetry Is instituted.
 In these pt. orotracheal intubation is defficult
b/c of restricted mouth opening , edema
arround mouth, lips and pharynx.
 Fibreoptic scope guided tracheal intubation is
the technique of choice in an anticipated
difficult airway.
 In these pt. mucosa is edematous and
hemorrhagic. So, the use of local anaesthetic
agents for awake fibreoptic intubation carried a
potential risk of toxicity due to direct absorption
of local anaesthetic drugs into the systemic
circulation.
CONT…
 Induction of anaesthesia with intravenous
agents carries an inherent risk of aponea and
subsequent failure to ventilate.
 Use of neuromuscular blocking drugs is
avoided to prevent cannot ventilate cannot
intubate (CVCI) situations.
 Tracheal intubation under inhalational
anesthesia is a good option when fibrescope
is not available.
 Pt. is premedicated with inj.Glycopyrolate &
inj.Midazolam.
 preoxygenation with 100% oxygen done for
five minutes
CONT…
 Inhalation anaesthetic agents is mixed with
oxygen.
 Direct laryngoscopy and intubation should be
done gently to avoid trauma to oro
pharyngeal tissues and subsequent bleeding.
 Tracheal tube should be well lubricated for a
smooth passage.
 In most cases, the proper tube size is 0.5 to
1.0 mm ID smaller than predicted for age
because of airway inflammation and edema
 Proper placement of tracheal tube is
confirmed by end tidal carbon dioxide
(EtCO2) and chest auscultation.
CONT…
 100% oxygen is given after inhalation agents
is switched off
 Patient remained hemodynamically stable
throughout the procedure.
 After become conscious and tolerating the
tracheal tube then shifted
 Laryngeal mask airway (LMA) has been
recommended in difficult airway but in
these patient it is not useful because it does
not prevent the risk of compression of airway
in the neck and it’s placement is difficult and
dislodgment common due to edematous and
swollen oral structures.
 CONT…
 Intubating laryngeal mask airway is a good
option but the risk of bleeding is more during
its placement.
 Nasotracheal intubation and tracheostomy are
contraindicated due to deranged coagulation
profile.
 Oral airway insertion is avoided to prevent
bleeding from fragile mucosa.
 Steroids has been used successfully as an
adjunct in oropharyngeal obstruction in
patients with leukemia.
CONT…
 The influence of corticosteroids on airway
patency in patients with pharyngeal swelling
may involve some direct vascular effects in
addition to well-established anti-inflammatory
effects (stabilization of vascular endothelium
and lysozymal membrane permeability and
suppression of phagocytosis).
 The direct vascular effects include increased
sensitivity of the vascular network to
endogenous vasoconstrictors and a competitive
blockade of estradiol receptors on the vascular
network.
T
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