CHRONIC MYELOID LEUKEMIA

Leukemias are malignant Neoplasms of the haematopoetic stem cells characterized by diffused replacement of bone marrow by Neoplastic cells with or without involvement of the peripheral blood.

➡️ Chronic leukaemia is a disease of insidious onset, affecting middle aged & young adults (CML)

🔅CLINICAL FEATURES:

  1. Gradually developing Anaemia
  2. Hepatosplenomegaly (In CML, a massive splenomegaly reaching upto the umblicus) Therefore, acute pain due to splenic infarction
  3. Bleeding tendencies
  4. Hypermetabolism
  5. Bone pain
  6. Juvenile CML: Lymph node enlargement, frequent infections, haemorrhagic manifestations & facial rash

🔅LAB. FINDINGS:

  • Thick and sticky & shows a wide buffy coat in the haematocrit tube
  • Anaemia – Moderate, normocytic normochromic
🔘 1 lakh - 5 lakh/cu.mm

🔘 Differential count reveals more mature forms in Peripheral smear, complete spectrum of granulocytes, myelocytes & band cells (50%)

🔘 Blast cell > 10% of leukocytes

🔘 Phases:

1. Chronic phase:

➡️ Myeloproliferative disorder – excessive proliferation of myeloid cells & mature segmented Neutrophils

➡️ Basophils upto 10% (feature of CML)

2. Accelerated phase:

  • ⬆️ Anaemia
  • Blast count: 10-20%
  • Basophils: 20%
  • Platelet: ⬇️ 1 lakh/µl
  • Leucocytosis

3. Blastic phase: Myeloid blast crisis in CML resembles AML >20%

  1. Cellularity: Hypercellular fat spaces replaced by Myeloid cells.
  2. Myeloid cells: ⬆️ M:E ratio
  3. Erythropoiesis: Normoblastic
  4. Megakaryocytes: Conspicuous, smaller in size
  5. Chromosomal abnormalities: The philadelphia chromosome is present in about 90% of the cases of CML

• neutrophil alkaline phosphatase (NAP) activity is markedly reduced in CML

🔅TREATMENT:

  • The standard treatment for chronic phase CML is a tyrosine kinase inhibitor (TKI) like imatinib oral therapy. Imatinib works by reducing the production of abnormal white blood cells.
  • Allogenic bone marrow transplants.
  • Interferon was once the best treatment for CML, but now, the tyrosine kinase inhibitors are the mainstay of treatment and interferon is rarely used. To treat CML, this drug is most often given as a daily injection (shot) under the skin. It may also be injected into a muscle or vein. It’s given for many years.
  • Chemotherapy: Today, chemo may be used to treat CML when the TKIs have stopped working. It’s also used as part of a stem cell transplant.
  • Others:
  • Splenectomy
  • Splenic irradiation
  • Leukapheresis

🔘 Splenectomy and splenic irradiation have been used in patients with large and painful spleens, usually in the late phase of CML. This is rarely needed in patients whose disease is well controlled.

🔘 For those patients unable to tolerate chemotherapy, leukapheresis is a viable option.

Dr. Mehnaz Memon🖊


References:

  1. Textbook of Pathology, Harsh Mohan; Internet articles
  2. Image source: Google

FRACTURE HEALING

There are four stages in the repair of a broken bone:👇🏻😀

Dr. Mehnaz Memon🖊


Summary💡

🔅What are the 4 stages of bone healing?

•Hematoma formation
•Fibrocartilaginous callus formation
•Bony callus formation
•Bone remodeling
🔅What is callus formation in bone healing?

Bone production begins when the clotted blood formed by inflammation is replaced with fibrous tissue and cartilage (known as soft callus). As healing progresses, the soft callus is replaced with hard bone (known as hard callus), which is visible on x-rays several weeks after the fracture.
🔅What foods heal bones faster?

•Iron helps your body make collagen to rebuild bone. It also plays a part in getting oxygen into your bones to help them heal.
Good sources: Red meat, dark-meat chicken or turkey, oily fish, eggs, dried fruits, leafy green veggies, whole-grain breads, and fortified cereals.
🔅How long does it take for a broken bone to fully heal?

•A broken bone and the surrounding soft tissue damage need a minimum of six to eight weeks to heal. However, depending on your general health and the condition of your bone and soft tissue, healing can take much longer.

References:

  1. Image: orthobullets.com
  2. Internet

Therapeutic Uses of Adrenaline

1) Vascular Uses:

(i) Hypotensive States: (Shock, Spinal Anaesthesia)

➡️ In case of anaphylactic shock or angioedema of Larynx or for bronchospasm attending drug hypersensitivity (Adrenaline + sub-class of gluco-corticoids) is recommended.

  • Put the patient in reclining position, administer oxygen at high flow rate
  • Inject adrenaline 0.5 mg (0.5 ml of 1 in 1000 solution for adult, 0.3 ml for child (6-12 years) & 0.15 ml for child (upto 6 years) i.m
  • Repeat every 5-10 min. in case patient does not improve.
  • This is the only life saving measure

(ii) Along with local anaesthetic:

➡️ Adrenaline 1 in 2,00,000 to 1 in 1,00,000 for infilteration, nerve block, spinal anaesthesia

🔅Effects:

  • Duration of anaesthesia prolonged
  • Systemic toxicity of LA ⬇️
  • Local bleeding minimized

(iii) Control of local bleeding: (Skin, mucous membrane eg. Epistaxis)

➡️ Compresses of adrenaline 1 in 10,000 can control arteriolar & capillary bleeding

2) Cardiac Uses:

🔅Cardiac Arrest (Drowning, Stokes-Adam syndrome)

👉🏻 Adrenaline is used to stimulate the heart, i.v infusion with external cardiac massage

3) Allergic disorders:

  • Adrenaline is a physiological antagonist of histamine which is an important mediator of many acute hypersensitivity reaction
  • Affords quick relief in urticaria, angioedema
  • Ineffective in delayed type allergy because histamine not involved.

4) Mydriatic:

👉🏻 The ester prodrug of adrenaline – Dipivefrine is an adjuvant drug for open angle glaucoma

5) Insulin hypoglycaemia:

👉🏻 Adrenaline can be used as an expedient measure but glucose should be given as soon as possible.

Dr. Mehnaz Memon🖊


References: Essentials of Medical pharmacology, KD Tripathi (7th Ed)

Internal Vs. External Resorption

What are internal and external resorption and the causes?

🔅The American Association of Endodontics defines resorption as, “a condition associated with either physiologic or a pathologic process resulting in a loss of dentin, cementum, and/or bone. Vital tissue is necessary for either external or internal resorption to occur.”

🔅By this definition, internal resorption is “a defect of the internal aspect of the root following necrosis of odontoblasts as a result of chronic inflammation and bacterial invasion of the pulp tissue.”

🔅External resorption is “resorption initiated in the periodontium and initially affecting the external surfaces of the tooth—may be further classified as surface, inflammatory, or replacement, or by location as cervical, lateral, or apical; may or may not invade the dental pulpal space.”

Difference between Internal Resorption Vs. External Resorption

Histology:

Clinical Case:

It may present initially as a pink-hued area on the crown of the tooth; the hyperplastic, vascular pulp tissue filling in the resorbed areas. This condition is referred to as a pink tooth of Mummery.
External Inflammatory lesion

Dr. Mehnaz Memon🖊


References:

  1. https://www.byendo-cupertino.com
  2. https://www.dentistryiq.com
  3. Image Source: Google

CANINE RETRACTORS

Canine retractors are springs that are used to move canine in a distal direction.

🔹ETIOLOGY:

  • slightly abnormal path of eruption
  • Local tooth displacements as in class 1 malocclusion
  • Buccal displacement is more common in crowded arch
  • Palatal displacement is more common in uncrowded arch & is associated with small or abnormal root formation of 2’s.

🔹CLASSIFICATION:

1. Based on location

  • Buccal (Buccally placed)
  • Palatal (Palatally placed)

2. Based on presence of helix or loop

  • Canine retractors with helix
  • Canine retractors with loop

3. Based on mode of action

  • Pull type
  • Push type

▪️BUCCAL CANINE RETRACTOR:

INDICATION: Is used where a buccally placed canine has to be moved palatally as well as distally.

TYPES:

• Self supporting or unsupported

• Supported

▪️SELF SUPPORTED BUCCAL CANINE RETRACTOR:

DESIGN & CONSTRUCTION

  • 0.7mm wire used
  • Coil lies just distal to the long axis of the tooth
  • Mesial arm-active arm, should be away from the tissue surface & should run parallel to the long axis of the canine
  • At about the middle third of canine a right angled bend is given to the active arm & is adapted on the mesial side of the canine.
  • Distal arm-retentive arm, adapted to the mesial aspect of 2 PM

ACTIVATION

👉🏻The spring should be activated by only 1mm. Only spring which is activated by closing the coil

👉🏻Distal activation- It is effected at the coil by bending the anterior limb over the round beak of a pair of spring forming pliers

👉🏻Palatal activation- It is undertaken in the anterior limb after it emerges from the coil

DISADVANTAGES

  • More flexible vertically than it is mesiodistally
  • Often stiff
  • Not indicated in lower arch

▪️SUPPORTED BUCCAL CANINE RETRACTOR:

DESIGN

  • Identical in design to unsupported but made from 0.5mm wire supported in tubing
  • More than twice as flexible as the standard retractor
  • Tubing imparts excellent vertical stability

ACTIVATION

  • Activation by 2mm
  • Adjustment made as described for self supporting BCR
  • More important not to bend the wire where it emerges from the tubing otherwise it may # at this site of stress concentration

▪️AN APPLIANCE TO RETRACT BUCCAL CANINE

Indication

  • Class 1 –crowding, upper canines are usually buccally placed
  • Class 2- to retract canine to provide space for overjet reduction
  • Active component
  • Supported buccal canine retractor
  • 0.5mm Sheathed in tubing.
  • Retention
  • adams clasp on 6|6.
  • Southend clasp on 1|1.
  • Baseplate
  • Normal full coverage
  • Anchorage
  • Spring pressure must be kept light

▪️SOLDERED AUXILIARY SPRINGS TO MOVE A CANINE PALATALLY

• It is possible to solder a spring{0.7mm wire} to the bridge of the adams clasp on a first molar.

• The spring can be used to tuck an outstanding canine

Advantages

  • Spring does not cross embrasure & does not compete with other wirework
  • Added easily to an existing appliance

Disadvantages

  • When the clasp is adjusted the spring position is also affected.
  • Difficult to add a spring to a clasp that is already carrying a soldered tube for facebow

▪️REVERSE LOOP BUCCAL RETRACTOR:

INDICATION

  • When the canine is placed in the line of the arch & has to be just distalized
  • Favored in shallow sulcus as in lower arch

DESIGN

  • 0.5mm ss wire used
  • The coil is placed as high as possible in the sulcus, but short of the final depth in relation to the space to which the canine has to be moved
  • Mesial arm-retentive arm, lies mesial to 2 PM
  • Distal arm-active arm. place at right angled bend to the active arm at about the cervical margin of the teeth & adapt it on the mesial side of the canine

ACTIVATION

  • Should be activated by not more than 1mm
  • Cut off 1mm of wire from the free end & re-forming it to engage the mesial surface of the tooth
  • Alternatively it can be activated by opening the loop by 1mm, it is better not to adjust at the loop in lower arch because this moves the active end of the spring occlusally so that secondary adjustment is required

DRAWBACKS

• It is stiff in the horizontal plane yet very unstable vertically

▪️An appliance to retract lower canine

Indication

Anterior crowding

Active component

Reverse loop buccal retractor

Retention

Adams clasp on 6|6

Baseplate

A lingual bar rather than acrylic in the incisor region

Anchorage

Clasped teeth

Teeth and alveolar process contacted by the appliance

▪️U loop canine retractor

INDICATIONS

  • Distal movement of canines
  • Where functional depth of sulcus is less

DESIGN

  • 0.7mm ss wire used
  • A U-loop approximately equal to the width of premolar is made
  • Mesial arm-active arm. At about the cervical margin of canine,a right angled bend is placed on the active arm to engage the mesial side of the canine
  • Distal arm-retentive arm, is adapted mesial to the 2 PM

ACTIVATION – Close the loops by 1mm or free end is cut by 1mm & it is readapted on to the mesial side

DRAWBACKS – Requires frequent adjustments

▪️AN APPLIANCE TO MOVE A CANINE OCCLUSALLY

Indication – Partially erupted canine

Active component – U loop canine retractor, engages a hook bonded to the buccal surface of the canine

Retention – Adams clasp on 6|6

Baseplate – As much palatal coverage as the canine position permits to offer max. resistance to the occlusally directed force

Anchorage – Acrylic in the palate

▪️Buccal acrylic appliance to retract lower canine

Indication – To overcome the dual problems of limited space for tongue & poor retention provided by adams clasp

Active component – Buccal canine spring 0.7mm

Retention – Lingually placed jacksons clasp on the first molar [to overcome the problem of limited undercut on the buccal aspect of lower molars, appliances have been described with clasping on the lingual aspect of molars BELL 1983]

Baseplate – 2 segments of acrylic 1-2mm from the buccal mucosa. These are connected in the midline by a heavy ss bar, {2mm x 1mm}oval in cross section lying close to buccal mucosa below the lower incisors

Anchorage – First molar, with minimal anchorage effect from acrylic

▪️PALATAL CANINE RETRACTOR:

INDICATIONS – In cases where canine is placed palatally & requires distal & buccal movement

DESIGN

  • 0.5mm ss wire used
  • coil is placed along the long axis of the canine
  • Distal arm-active arm, placed mesial to canine
  • Mesial arm-retentive arm, is bent at rt angle & extends up to mesial aspect of first molar where a retentive tag is placed

ACTIVATION – By opening the coil by 2-3mm where the active arm emerges out of the coil

▪️CONCLUSION:

• For canine retractors whether buccal or palatal to be successful, it is important for the canines to be mesially angulated prior to treatment

• If the canine is normal & distally angulated prior to treatment, a removable canine retractor will cause an unsightly distal angulation of the canine at the completion of retraction which in turn will take a long time to correct with fixed appliance

• For this reason, use of removable appliance for canine retraction is declining as fixed appliance have greater control over tooth movement


References:

  1. Textbook of Orthodontics The Art and Science 6th Edition By Bhalajhi; Internet
  2. Image Source: Google

BULLOUS PEMPHIGOID

A chronic, autoimmune, sub-epidermal blistering skin disease that rarely involves mucous membrane.

🔹Clinical Features:

Age: Elderly (>60 years)

Skin Lesions:

  • Generalised non-specific rash, commonly on Limbs.
  • Appears urticarial/eczematous; persist for several weeks to months.
  • Vesicles & bullae arise in prodromal skin lesion as well as Normal skin.
  • The blisters are thick walled and don’t rupture easily.
  • ruptured blisters are usually sensitive and painful, have raw eroded area which heals rapidly.

Oral Manifestations:

Vesicles appear gingivally👇🏻

Erythematous & desquamate as result of minor frictional trauma

👉🏻 Oral lesions comprise of bullae/vesicle that rupture to form erosions and ultimately leave out ulcerations

👉🏻 Other sites:

  • Buccal Mucosa
  • Tongue
  • Floor of the mouth
  • Palate

🔹Diagnosis:

Apart from evaluating history, clinical presentation, histopathological analysis is carried out followed by direct immunofluorescence study for the differential diagnosis and confirmation of the condition.

👉🏻Histopathology:

  • Acanthotic mucosa
  • Subepidermal non-specific vesicles with fibrous exudate

👉🏻Direct immunofluorescence is found to be the gold standard test. Deposition pattern of different types of immunoreactants differentiates the various immune-mediated diseases. Direct immunofluorescence shows presence of IgG and C3 deposits along the basement membrane zone.

©️jiaomr.in
👉🏻Electron Microscopy: In bullous pemphigoid (BP), the 180 kD antigen (BPAG2) was shown by immuno-EM to be a transmembrane molecule and to possess an autoantibody binding site outside the cell, suggesting a major pathogenic role for the BPAG2 in blister formation.

🔹Differential Diagnosis:

• Mucous membrane pemphigoid can be differentiated from BP by its predominant involvement of mucosal surfaces and positive Nikolsky’s sign.

• Lichen planus pemphigoides is clinically differentiated by the presence of lichen planus lesions in addition to tense blisters.

• Nikolsky’s sign is present in case of pemphigus and cicatricial pemphigoid, but not in the case of BP.

🔹 Treatment:

👉🏻Treatment is based on the degree of cutaneous and oral involvement. Mostly, topical steroid (clobetasol propionate) gives satisfactory result in case of smaller area of skin involvement, whereas larger area of skin involvement and recurrent cases are treated satisfactorily with systemic steroids and immunosuppressive agents.

👉🏻Recommended dosage for oral prednisolone is 0.3–1.25 mg/kg body weight/day, controls disease within 1–2 weeks, followed by which the dose is tapered. Dexamethasone (100 mg in 500 mL 5% dextrose i.v. over 2–3 h for three consecutive days) is the preferred steroid for pulse therapy, either administered alone or in combination with cyclophosphamide.

Other drugs for treating BP include new antibody modulators, rituximab 375mg/m2weekly over 4 weeks and omalizumab subcutaneously 300–375 mg for every 6 weeks.

👉🏻Higher doses of systemic corticosteroids seem to be associated with higher mortality rates, which led to the addition of corticosteroid-sparing agents to the treatment of BP. The most frequently used immunosuppressive agent is azathioprine (0.5–2.5 mg/kg body weight/day). Others being cyclophosphamide, methotrexate, cyclosporine A, combination tetracycline/minocycline along with nicotinamide, and more recently, mycophenolate mofetil, a DNA synthesis inhibitor, and methotrexate, a folate antagonist.

👉🏻IVIg – A dose of 1–2 g/kg for five consecutive day cycle of 0.4 g/kg/day, although a 3-day cycle may be used in cases that are nonresponsive to conventional therapy.

Dr. Mehnaz Memon🖊


References:

  1. http://www.jiaomr.in/article.asp?issn=0972-1363;year=2018;volume=30;issue=4;spage=432;epage=435;aulast=Aparna
  2. https://www.cidjournal.com/article/S0738-081X(00)00178-4/abstract
  3. Shafer’s Textbook of Oral Pathology, 7th edition
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