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Myelofibrosis

Heri Sutrisno
SMF Penyakit Dalam
RSU WZ Johannes Kupang
Introduction:

 Myeloproliferative disorders describes a group


of conditions characterized by clonal proliferation
of one or more haemopoietic components in the
bone marrow & sometime in the liver & spleen.

Division of Hematology & Oncology, Dept. of Internal Medicine


Introduction

Myeloproliferative disorders :

1 Polycythemia Vera ( PV )

2 Essential Thrombocytemia (ET )

3 Chronic Myelocytic Leukemia

4 Myelofibrosis ( MF )

Division of Hematology & Oncology, Dept. of Internal Medicine


Hematopoetic stem cel

Prekursor Prekursor Prekursor Fibrosis


granulosit eritrosit magakariosit reaktif

CML PV TE MF

AML

Division of Hematology & Oncology, Dept. of Internal Medicine


Introduction:

• Other designations (angiogenic myeloid


metaplasia,myelofibrosis with myeloid metaplasia).

• A clonal disorder of hematopoeitc progenitor cell of


unknown etiology characterized by marrow
fibrosis,myeloid metaplasia ,with extramedullary
hematopoiesis & hepatosplenomegally.

• There is over production of collagen.

Division of Hematology & Oncology, Dept. of Internal Medicine


Myelofibrosis

Bone marrow fibrosis

Splenomegaly & Hepatomegaly Characterized by

Blood smear :
leuco-erithroblastic
( immature leucocyte & erythrocyte ),
poikilositosis: tear-drops cells

Division of Hematology & Oncology, Dept. of Internal Medicine


Etiology:

• The etiology is unknown.

• No specific cytogenitc abnormality has been identified.

Division of Hematology & Oncology, Dept. of Internal Medicine


Pathogenesis
Platelet Derived Growth Factor (PDGF) secretion
& other cytokine >>

bone marrow fibrosis

extramedullary hematopoesis
in the spleen, liver, limph-node

(reactivation of the mesenchymal tissue


which was active for hematopoesis in foetus)

Division of Hematology & Oncology, Dept. of Internal Medicine


Clinical features:

• Age > 50 years old


• No specific sign or symptoms.
• Most patients are asympotomatic at presentation
&usually recognized by splenic enlargement &/or
abnormal blood count .
• Blood smear revels the characteristic feature of
extramedullary hematopiesis.
• Mild hepatomegally.
• LDH&Alkaline phosphatase can be high.
• BM aspirate will give dry tape.

Division of Hematology & Oncology, Dept. of Internal Medicine


Division of Hematology & Oncology, Dept. of Internal Medicine
Diagnosis:

• The diagnosis is by exclusion.


• Peripheral blood film (teardrop-shaped red
cells,nucleated red cells,myelocytes,and promyelocytes)
establishes the presence of extramedullary
hematopoeisis.
• Bone marrow is usually not aspirable due to increase
marrow reticulin.
• Marrow biopsy:hypercellularity marrow,with trilineage
hyperplasia&in particular megakaryocytes.

Division of Hematology & Oncology, Dept. of Internal Medicine


Clinical Sign
In the late phase ( bone marrow failure due to fibrosis ):

1 Severe Anemia  transfusion

2 Severe thrombocytopenia  bleeding

3 Spleenomegaly >>  early satiate

4 Spleen Infarction  pain/ shock (surgery)

5 Cachexia

Division of Hematology & Oncology, Dept. of Internal Medicine


Clinical Sign

6 Bone pain (lower extremity)

7 Hematopoesis in the liver  portal hypertension


(ascites & esophageal varices)

8 Abnormal myelopoesis in epidural cavity


Transversal Myelitis

Division of Hematology & Oncology, Dept. of Internal Medicine


Laboratorium

1. Mostly anemia
2. Leucocyte could be < , normal, atau >
 (sometimes 50.000 / mm3)
3. Thrombocyte count is vary
 Abnormal thrombocyte (giant)
4. Blood smear: poikilositosis,tear-drop cell
(abnormal erythrocyte)

Division of Hematology & Oncology, Dept. of Internal Medicine


Laboratorium

Teardrop cells & nucleated red blood cells


Division of Hematology & Oncology, Dept. of Internal Medicine
Laboratorium

• immature erythrocyte & myeloid cell


(mieloblast / promielosit)
• Bone Marrow: dry-tap, initial phase:
hypercellular bone marrow
• Early phase: reticulin, late phase: collagen
• Chromosome abnormality (-)

Division of Hematology & Oncology, Dept. of Internal Medicine


Laboratorium

Myelofibrosis of the bone marrow. Total replacement of marrow precursors


and fat cells by a dense infiltrate of reticulin fibers and collagen (H&E stain).

Division of Hematology & Oncology, Dept. of Internal Medicine


Laboratorium

Reticulin stain of marrow myelofibrosis. Silver stain of a myelofibrotic


marrow showing an increase in reticulin fibers (black-staining threads).

Division of Hematology & Oncology, Dept. of Internal Medicine


Division of Hematology & Oncology, Dept. of Internal Medicine
Differential Diagnosis

MF

Leuco-erythroblastic :
Found in severe infection, Bone Marrow Fibrosis :
severe inflammation, bone Found in metastatic
marrow infiltration (no tear- carcinoma, Hodgkin,
drops cells & giant Hairy cell leukemia
thrombocyte)

Division of Hematology & Oncology, Dept. of Internal Medicine


Complication:

• Chronic disease of a median survival of 5years.


• The patients are prone to deep-seated tissue infections
particularly of the lung.
• Can transform to accelerated phase with constitutional
symptoms and marrow failure.
• In 10% of patients the disorder terminates in a rapidly
progressive form of acute leukemia for which therapy is
usually is ineffective.

Division of Hematology & Oncology, Dept. of Internal Medicine


Management
No specific treatment.
1. Anemia  transfussion
2. Androgen (Oxymetholone 200 mg p.o/ day,
or testosterone)
3. Splenectomy, if the patients experienced
pain, severe thrombocytopenia, or
transfussion dependent >>
4. Interferon Alfa, 2-5 MU, Sub-Cutaneous,
three times a week
5. Allogenic bone marrow transplantation
( young patients )  50 % long-term survival

Division of Hematology & Oncology, Dept. of Internal Medicine


Prognosis

Median survival: 5 years after first diagnosis

Late phase: General weakness, liver failure,


bleeding due to thrombocytopenia

Several cases transform into Acute Myeloid Leukemia

Division of Hematology & Oncology, Dept. of Internal Medicine