Plasma Cell Myeloma

Oleh :
Gst. Ayu Laksmi Dewi Saputri
 Plasma cell myeloma is a neoplastic plasma cell disorder that usually presents
after the fifth decade of life;
 It is rarely described in younger population especially under 30 years of age

 In approximately 2% of plasma cell myeloma, the morphology of the

neoplastic cells is highly pleomorphic, quite anaplastic, and may resemble
metastatic tumor cells
 PCM is primarily and predominantly a disease of the bone marrow characterized by
neoplastic plasma cell proliferation associated with a monoclonal immunoglobulin
(M protein) in serum or urine.

 The diagnosis is based on clinical, radiological, laboratory, and cyto-histological

findings

 The bone marrow is used for both qualitative and quantitative assessment using
immunophenotyping

 Based on the cytomorphology, myeloma cells are classified into 4 types: mature,
immature, pleomorphic, and plasmablastic
 A myeloma cell is a post-germinal centre plasma cell (long-lived memory B-
cell) that has undergone immunoglobulin gene recombination, class switching
and somatic hypermutation, and homes to the bone marrow.

 Therefore, classical clinical features of end organ damage, bone marrow

plasmacytosis (≥10%), osteolytic bone lesions, and hypergammaglobulinemia
in serum protein electrophoresis (monoclonal spike) are some of the findings
in keeping with the diagnosis of Plasma Cell myeloma
 This include clinical assessment of myeloma-related end-organ impairment in
the presence of serum M-protein and bone marrow plasma cells.
 The end organ damage is defined using both the classic “CRAB” criteria of
1. hypercalcemia (serum calcium >11.5 mg/dl)
2. renal failure (defined by serum creatinine > 2.0 mg/dl, or estimated
creatinine clearance < 40 ml/minute with no other etiology),
3. normochromic normocytic anaemia with a hemoglobin value < 10 g/dL (or a
hemoglobin value < 2 g/dL below the lower limit of normal), or bone lesions
(lytic lesions ,severe osteopenia, or pathological fractures) and additional
criteria including recurrent bacterial infections (>2 in 12 months)
4. amyloidosis, or symptomatic hyperviscosity
 The etiology of Plasma cell myeloma is poorly understood but studies have
shown genetic factor as one of the predisposing factors. Occasional reports in
family members and spouses with HLA-Cw2 and Cw5 have been recorded
(about 3-5% familial tendency).
 In many instances, patients have had surgery, with complete or partial tumor
removal, as part of the diagnostic procedure. Apart from the diagnostic
approach, the indication of surgery are fixation of fractures, decompressive
laminectomy, or spine stabilization. The introduction of modern spinal
fixation and stabilization methods, such as vertebroplasty and kyphoplasty,
allows for a surgical solution for patients who develop vertebral fractures,
vertebral instability, neurological complications, or a combination of these
MRI findings

Expansile focal mass

Multiple focal masses in

the appendicular skeleton

Diffuse marrow
involvement

Multiple compression
fractures in the patient
with no known malignancy
 Case Report

Identitas Pasien

Nama : Ketut Nami

Umur : 57 tahun

No. RM : 22059350

Pekerjaan : Ibu Ruma Tangga

Asuransi : BPJS

Tanggal MRS : 17/12/2022

Ruang Rawat : Angsoka 305.3

Lanjutan..

 Keluhan utama : kelemahan anggota gerak bawah

sejak 2 minggu SMRS
jatuh terpeleset dikamar mandi 1 bulan yang lalu (5/11/2022)
2 minggu pasca jatuh pasien mulai mengeluh kelemahan pada kedua kakinya, hingga
pasien sulit berjalan mandiri
saat ini pasien tidak dapat berdiri maupun berjalan BAB dan BAK tidak terasa.
  Pemeriksaan Fisik

Primary survey

Airway : clear + C-spine control

Breathing : spontan, RR : 16x/menit SO2 99% room air

Circulation : stabil, TD 100/80 mmHg, HR : 70x/menit, CRT < 2 detik

Disability : GCS E4V5M6

Exposure : suhu 36.8 0C

 Status lokalis

Regio Thoracolumbal Region:

 Look : Swelling (-), deformity (-), bruise (-)

 Feel : Midline tenderness (-), hypoesthesia (+) below level T10, anesthesia (-)

 Move: 55555/55555

00000/0000
 Terapi :

Parasetamol 4x500mg PO

Neuroprotector
Diagnosis :
Immobilization with TLSO
Pathological Fracture CV Th8 ec
P/MRI Thoracolumbal + Contrast
susp Spondylitis TB dd Pyogenic

Infection P/Decompression + Stabilization + Fusion + Biopsy + Culture

Assessment :
Post Decompression - Stabilization -
Fusion + Biopsy + Culture (26/12/2022)
ec. Pathological Fracture CV Th8 ec
susp Spondylitis TB dd Pyogenic
Infection
Diskusi

 Pasien perempuan usia 57 tahun dengan keluhan utama : kelemahan anggota

gerak bawah secara tiba-tiba, Riwayat terjatuh 2 minggu sebelumnya.
 Tanda klinis pada kompresi kord akut ialah nyeri, kelemahan motorik, perubahan
sensoris, disfungsi otonom dan instabilitas mekanik dari spinal kord. Sekitar 68% kasus
terjadi pada thoracic spine, 7,5% pada lumbar dan 22,6% pada cervical

 Pathological Fracture CV Th8 ec susp Spondylitis TB dd Pyogenic Infection

 Nyeri semakin parah merupakan indikasi adanya fraktur patologis dari spine.
 Lesi pada atau diatas dari konus medularis bisa menjadi paralisis simetri pada
ekstremitas bawah dan kelemahan fleksor.
Fraktur kompresi multipel, osteoporosis pada tulan vertebra dan
jaringan paraspinal merupakan gambaran MRI pada multipel
myeloma.
- peningkatan formasi rouleaux dengan beberapa sirkulasi sel
neoplastic dengan “blastoid”
- penyebaran aspirasi sumsum tulang infiltrasi padat dengan
klaster kohesiv/lembaran dipenuhi sel neoplastic pleomorfik,
banyak yang menunjukkan morfologi anaplastic
- sel dengan morfologi anaplastic yang sangat besar dengan
sitoplasma dan ditandai kontur secara langsung
- beberapa sel menunjukkan morfologi blast-like
- sel plasma scattered dengan hiperkromatik nuclei dengan
frekuensi tertandai bentuk mitotic.
 Decompression - Stabilization - Fusion + Biopsy + Culture (26/12/2022) ec.
Pathological Fracture CV Th8 ec susp Spondylitis TB dd Pyogenic Infection
 In many instances, patients have had surgery, with complete or partial tumor
removal, as part of the diagnostic procedure. Apart from the diagnostic
approach, the indication of surgery are fixation of fractures, decompressive
laminectomy, or spine stabilization
 Standar terapi : Bortezomib, lenalidomide, and dexamethasone (VRd)
Simpulan

 Plasma cell myeloma (PCM) adalah penyakit clonal maligna yang ditandai dengan adanya proliferasi
dari sel plasma, yang merupakan penyebab tersering kedua setelah leukemia pada kasus malignansi
hematologic
 Plasma cell myeloma (PCM) sering terjadi pada tulang utamanya pada kolumna spinal
 Tanda klinis pada kompresi kord akut ialah nyeri, kelemahan motorik, perubahan sensoris, disfungsi
otonom dan instabilitas mekanik dari spinal kord. Sekitar 68% kasus terjadi pada thoracic spine,
7,5% pada lumbar dan 22,6% pada cervical
 Nyeri semakin parah merupakan indikasi adanya fraktur patologis dari spine.
 Lesi pada atau diatas dari konus medularis bisa menjadi paralisis simetri pada ekstremitas bawah dan
kelemahan fleksor.
 Fraktur kompresi multipel, osteoporosis pada tulan vertebra dan jaringan paraspinal merupakan
gambaran MRI pada multipel myeloma.
 Standar terapi : Bortezomib, lenalidomide, and dexamethasone (VRd)
 Terimakasih