CASE PRESENTATIONACUTE LYMPHOID LEUKEMIA

(AIRWAY MANAGEMENT)

By: Dr. Govind K. Goyal

Moderator: Dr. Ramesh C. Sunar

HISTORY

Name- Raghavendra s/o DeenanathAge- 15 YrsSex- MaleResidence- 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/m3, 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. FibrinogenII. ProthrombinIII. Tissues plasminogen factor,ThromboplastinIV. CalciumV. Proaccelerin , labile factorVI. Not namedVII. Proconvertin, Stable factorVIII. Antihemophilic factor AIX. Antihemophilic factor B Or Christmas factorX. Stuart-Prover factorXI. Plasma thromboplastin antecedentXII. Hageman factor, Glass contact factorXIII. Fibrin stablizing 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%) Basophil (0.2-1%)

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

FUCTIONBlood 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 & BSickle cell anaemia VwdHerediatary spherocytosis DICThalassemia Aplastic TTPPNH Polycythemia ITPMegaloblastic anaemia Myelophthisic HUS Pernicious anaemia Leukemia Thrombastheniairon 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 Subtype

% of Cases FAB Subtype Cytogenetic

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.

THANKS