slide1 n.
Download
Skip this Video
Loading SlideShow in 5 Seconds..
Hairy Cell Leukemia HCL PowerPoint Presentation
Download Presentation
Hairy Cell Leukemia HCL

Loading in 2 Seconds...

play fullscreen
1 / 39

Hairy Cell Leukemia HCL - PowerPoint PPT Presentation


  • 205 Views
  • Uploaded on

Hairy Cell Leukemia HCL. Hairy cell leukemia is a chronic Lymphoprolifrative disorder. in 1952 was recognized. Leukemic reticuloendotheliosis. Lymphoid myelofibrosis. Reticulum cell leukemia. HCL Neoblastic B Lymphocyte. carry surface immunoglobulins of only one

loader
I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.
capcha
Download Presentation

PowerPoint Slideshow about 'Hairy Cell Leukemia HCL' - verlee


An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.


- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -
Presentation Transcript
slide2

Hairy cell leukemia is a chronic

Lymphoprolifrative disorder.

in 1952 was recognized.

Leukemic reticuloendotheliosis.

Lymphoid myelofibrosis.

Reticulum cell leukemia.

slide3

HCL

Neoblastic B Lymphocyte.

carry surface immunoglobulins of only one

light chain type Often isotype is of Gama class

or Multiple heavy chain.

H.cell express receptors for Fc portion of IgG. IgM

HCL reactivity with antibodies to CD19 .CD20, CD22 , CD25 , CD103 and MLA-DR.

PCA-1 is detected in HC & Plasma cell.

( CD11c, CD25 and CD103 )

slide4

The hairy cells are 10 to 15 mm in diameter with pale blue cytoplasm and a nucleus with a loose chromatin structure and one or two indistinct nucleoli.

slide5

Hairy Cell Leukemia

In PBS

Pancytopenia

Lymphocytosis

Hairy cell

Infection

Middle age

Male / female

slide9

Symptomatology:

Patients have fatigue due to anemia, fever, weight loss, and/or abdominal discomfort.

Sometimes the disease is diagnosed when patients have infection secondary to granulocytopenia or monocytopenia.

The only consistent physical findings are slight to marked splenomegaly (75 to 80% of cases)

slide10

Bone marrow aspiration is usually inadequate owing to increased reticulin, collagen, and fibrin deposition; bone marrow biopsy is usually necessary.

The biopsy demonstrates increased cellularity with a diffuse or occasionally patchy infiltrate with hairy cells.

The infiltrate is loose and spongy, with pale-staining cytoplasm surrounding bland, monotonous round or ovoid nuclei.

slide11

Etiology

No clear etiology

Benzene exposure

Radiation

slide14

The cytopenias are due to a combination of :

*Bone marrow production failure caused by leukemic infiltration.

*Hypersplenism. (increased destruction)

Marrow failure may be due in part to*inhibitory factors (e.g., tumor necrosis factor) produced by the leukemic infiltrate,

slide15

Laboratory abnormalities in HCL is as follows:

Anemia — 85%

Thrombocytopenia — 80%

Neutropenia — 80%

Monocytopenia — 80%

Azotemia — 30 %

Abnormal liver function tests — 20%

Hypergammaglobulinemia — 20%

Leukocytosis (WBC >10,000) 10 to 20%

slide17

The mucosal lymphocyte antigen, CD103:

is a sensitive marker for HCL.

is a member of the integrin family The presence of CD103 , when coexpressed, is highly suggestive of HCL

slide18

In HCL:

Vasculitis.

Arthritis.

Polyarthritis nodosa.

Systemic Vasculitis.

Many patients complaint:

Arthitis or arthralgia.

slide19

Diagnosis:

Examination of the blood often suggests the diagnosis of HCL.

In addition to the cytopenias the peripheral blood film usually demonstrates relative or absolute lymphocytosis, composed of cells with cytoplasmic projections, giving rise to the name hairy cell leukemia

slide20

patients often experience repeated infections and, more rarely, a

systemic vasculitis

Resembling polyarteritis nodosa

slide22

TRAP test

Hairy cells exhibit a strong acid phosphatase (isoenzyme 5).

Cytochemical reaction in more 95% of cases, a reaction that is resistant to the inhibitory effect of tartaric acid (TRAP).

slide23

Hairy cells expressCD19, CD20, CD22CD25 , CD11C , CD103 , FMC7, but not CD21 or CD5.

Cell-surface immunoglobulins can be immunoglobulin G (IgG) or immunoglobulin A (IgA),

The cells demonstrate a κ or λ light-chain phenotype

slide24

Clinical presentation

*Abdominal fullness or discomfort dueto *Splenomegaly, which may be massive.

*Systemic complaints (eg, fatigue, weakness, and weight loss).

*Bruising and bleeding

*Recurrent infections, which may be life- threatening, secondary to granulocytopenia and monocytopenia.

*Splenomegaly or cytopenias

slide25

Annexin A1 : HCL has a gene expression profile that is distinct from that of other B cell lymphomas, including overexpression of the ANXA1 gene (annexin A1)

slide26

DIFFERENTIAL DIAGNOSIS

1-Small B cell lymphoproliferative disorders. associated with splenomegaly .

2-Chronic lymphocytic leukemia (CLL),

3-Prolymphocytic leukemia .

The abnormal B cell in CLL usually stains positively for CD5, B cell CLL is usually negative for CD103, prolymphocytic leukemia present with splenomegaly, marked elevation of the white blood cell count, and the characteristic morphology of the prolymphocytes

slide27

Renal insufficiency in HCL due to:

Infiltration

Bleeding

Infections

Immune Complex

Protein excertion

Light chain deposition ( Nephrotic Syndrome)

Acquired Fanconi’s syndrome

( Proximal renal tubules)

slide28

A Microbicidal defect of the neutrophils and

aggregation defect of platelets have been reported in HCL

slide29

Patients with HCL have a predilection to develop tuberculosis , atypical mycobacterial infections, or fungal infections,

perhaps related to the severe monocytopenia that is characteristic of this disorder.

Pneumonia and

septicemia are common causes of death in HCL.

slide30

(Treatment)

Splenoctomy

Interferon,

2-CDA,

pentostatin

Cladribine

Granulocyte colony-stimulating factor (G-CSF)

Rituximab

Anti-CD20 antibody)

Anti-CD22 antibody (BL22)

Anti-CD25 antibody (LMB-2)

slide31

Splenectomy, often used in the past as the first treatment of most patients with complications from HCL, temporarily improves blood cell counts in two thirds of patients, usually within 1 to 4 weeks, but does not decrease the infiltration of hairy cells in the marrow or reduce the incidence of infections.

slide32

Treatment

A small proportion (<5%) of patients with HCL do not require therapy.

Splenectomy, often used in the past as the first treatment of most patients two thirds of patients, usually within 1 to 4 weeks, but does not decrease the infiltration of hairy cells in the marrow or reduce the incidence of infections.

Splenectomy is recommended mainly for patients with splenic infarcts or massive splenomegaly.

slide33

2-Chlorodeoxyadenosine (2-CDA), an adenosine analogue deaminase , produces complete remissions in more than 90% of HCL patients with a single course of 0.1 mg/kg/day for 7 days by continuous intravenous infusion.

slide34

Effect of 2-CDA

2- CDA 0.1 mg/kg body weight per day by continuous intravenous infusion for seven days

The complete and partial remission rates were 91%. The overall survival was 96%

The relapse rates for patients achieving CR ( after 10Y) were 16 % .

slide35

Cladribine (2-CdA)

Cladribine accumulates in lymphoid cells presumably because they are rich in deoxycytidine kinase. This enzyme phosphorylates cladribine, creating a deoxynucleotide that cannot readily exit the cell. The net effect is lymphotoxicity.

slide36

Cladribine and Pentostatin:

In one study of 219 patients followed for a median period of 12.5 years, overall response rates (100 versus 96 percent), rates of complete remission

10-year survivals (100 versus 96 percent) were similar for initial treatment with either cladribine or pentostatin, respectively.

slide37

Side effects:

Both Cladribine and Pentostatin produce prolonged immunosuppression

Opportunistic infections, Herpes zoster

slide38

Treatment Relapsed HCL

Retreatment with a second cycle of Cladribine or Pentostatine leads to a second complete remission in up to 70 percent of patients