Monday, March 23, 2009

Identification data - osteolytic bone lesion



A 70 yeard old lady, came to Casualty with the complains of right upper quadrant abdominal pain and back pain for 4 months. She also has history of recurrent fever and easy fatigubility. On the microscopic examination of her blood sample, numerous rouleaux formations were noted. The above picture is her skull radiograph.

a) State ONE (1) abnormality seen in this radiograph shown.
b) State the most likely diagnosis.
c) State ONE (1) cause of renal impairment in this patient.
d) State the plasma protein band which will be elevated prominently by electrophoresis in this case.
e) State the treatment for this condition.


Galata Tower,menara yang telah digunakan Sultan Muhammad untuk melihat seluruh Constantinopole sebelum merancang untuk menakluknya

Answers :

a) Multilytic/multiosteolytic lesion (kalau ikut kata prof zabidi, 1st kita describe apa yang kita nampak, so bone selalu nampak putih kan, kalau hitam kt kawasan bone, maybe ada lytic lesion, logic thinking)

b) Multiple myeloma

c) Bence-Jones protein, hypercalcemia, dehydration

d) Gamma band

e) Chemotherapy

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Notes about multiple myeloma

MM or myelomatosis is a neoplastic proliferation characterized by plasma cell accumulation in the bone marrow, the presence of monoclonal protein in the serum and/or urine and related tissue damage.

98% occur over the age of 40 years old, peak : 7th decade (60-70y/o)

Myeloma cell - is a post-germinal centre plasma cell that has undergone Ig class switching and somatic hypermutation and secretes the paraprotein that is present in the serum.

Plasma cell naturally home to the bone marrow, but the tumor cell retained this characteristic

Diagnosis
Depend on 3 principal findings :
  • monoclonal protein in serum and/or urine
  • increase plasma cells in the bone marrow
  • related organ or tissue impairment eg bone disease, renal impairment, hyperviscosity, recurrent infection, anaemia, hypercalcemia, amyloidosis
Note : if the bone marrow plasma cell count is >10%, but NO evidence of tissue damage, the disease is termed asymptomatic or smouldering myeloma.

Clinical features
  1. Bone pain (esp backache)
  2. Features of anaemia : lethargy, weakness, dyspnoea, pallor, tachycardia
  3. Features of renal failure/hypercalcemia : polydipsia, polyuria, anorexia, vomiting, constipation, mental disturbance
  4. Recurrent infections (due to deficient antibody production, abnormal cell-mediated immunity, and neutropenia)
  5. Abnormal bleeding tendency (myeloma protein interfere with platelet function and coagulation factors)
  6. Amyloidosis (5% of cases) with features such as macroglossia (large tongue), carpal tunnel syndrome, diarrhoea
  7. Hyperviscosity syndrome (2% of cases) with purpura, hemorrhages, visual failure, CNS symptoms, neuropathies, and heart failure.
Lab Findings

1. Serum and urine should be screened by Ig electropheresis
  • The paraproteins are Ig G , Ig A , light chain only, Ig D, Ig E
  • Free light chains are filtered from the serum into the kidney; therefore not detectable in serum using electrophoresis
  • Level of free light chains can be measured with a specific antibody
  • Normal serum Ig levels (IgG, IgM, IgA) are low -> immune paresis
  • Urine contains free light chains -> Bence-Jones proteins
2. Normochromic normocytic OR macrocytic anaemia
  • Rouleaux formation is marked
  • Neutropenia and thrombocytopenia (in advanced disease)
  • Abnormal plasma cells in blood film
3. Increase ESR and C-reactive protein (CRP)

4. Increase plasma cells in the bone marrow (usually > 20%) often with abnormal forms

5. Radiological investigation (ada bone lesion) :
  • osteolytic areas without evidence of surrounding osteoblastic reaction or sclerosis in 60% of pts OR generalized osteoporosis in 20% cases.
  • 20% have no bone lesions
  • pathological fracture or vertebral collapse are common
  • osteoclastic lesions are caused by osteoclastic activation resulting from high serum levels of RANKL (receptor activator of nuclear factor-kB ligand)
  • RANKL produced by plasma cells and bone marrow stroma, which binds to activatory RANK receptors on osteoclast surface
6. Serum Ca2+ increased (serum ALP is normal except in pathological fracture)

7. Serum creatinine increased
  • proteinaceous deposits from heavy Bence-Jones proteinuria, hypercalcemia, uric acid, amyloid, and pyelonephritis may all contribute to renal failure
8. Decrease serum albumin (advanced disease)

9. Serum B2-microglobulin is often increased and useful indicator of prognosis
  • level <4mg/l> good prognosis
Treatment : baca sendiri ye, sebab rasanya tak penting sgt, tau chemotherapy cukup.

reference : Essential hematology (pg 216-223)

5 comments:

Ibnu Abas on March 23, 2009 at 7:04 PM said...

wah!! pasni boleh la jadi lecturer ye azizi.. sambil2 tu boleh cari downline Amway nnt..

x gitu gg??

Ibnu Shaukani on March 23, 2009 at 7:20 PM said...

yg jd lecturer tu insyaAllah =P

weh, ak takde kaitan dgn amway ok..

haidarrealm on March 23, 2009 at 9:03 PM said...

salam..nk tanye,ape lg differential bg osteolytic lesion??

CT scan said...

nak share ni copy frm wikipedia~

A mnemonic sometimes used to remember the common tetrad of multiple myeloma is CRAB: C = Calcium (elevated), R = Renal failure, A = Anemia, B = Bone lesions.

all the Best!=D

`Aainaa Nur Raihana Abdullah on March 26, 2009 at 10:34 PM said...

mr azizi.. lecture ni ada.. cuma x sedetail ni.. cuba hang buka lymph patho by dr msms.. haihz~ :D

 

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