Case 3

Male, 68 years old

Onset of the disease : October 2007 pallor of skin, fatigue, enlarged cervical lymph nodes

 January 2008
Symptoms and signs : severe fatigue, intense pallor of skin, jaundice NO fever, sweats, weight loss Clinical examination : pallor, jaundice, enlarged cervical and supraclavicular lymph nodes 2 cm, rubbery consistence, moveable

Blood tests
Cell counts : Hb=6 g/dl, Ht=15%, MCV =106 fL, WBC=13.800/mmc with Mi 1, S 70, B 1, Ly 16, Mo 12; PLT=443.000/mmc. Reticulocytes 12% Blood smear : slight anysocytosis, slight poikylocytosis microsferocytes, hypochromia. ESR = 70 mm per hour

 Coagulation normal Biochemistry : normal BUN, creatinine; normal liver enzymes; TB 4 mg/dl, UB 3 mg/dl, CB 1 mg/dl, LDH 400 U/l, uric acid 10 mg/dl, iron 115 g/dl. Antiglobuline test direct IgG+C3d positive 4+,IgG positive 3+; indirect positive 2+.

What is the diagnosis ? 1. AIHA 2. DIC 3. TTP 4. Hodgkin disease/NonHodgkin lymphoma 5. Hereditary spherocytosis 6. Evans syndrome

 AIHA

Is AIHA idiopathic or secondary ?

Screening for infectious disease

AgHBs- negative Ac anti HCV negative HIV negative Mycoplasma pneumoniae negative Chlamydia negative CMV - negative

Screening for autoimmune disease

Ac anti DNA negative Ac anti SM negative Ac anti Ro and La - negative

Screening for chronic lymphoproliferative disorder

CT scan cervical, thorax, abdominal Bone marrow aspiration and biopsy Flow-cytometry from peripheral blood and bone marrow Lymph node biopsy Immunohistochemistry on lymph node and bone marrow

 What do u choose for this patient ?

 Lymph node biopsy Bone marrow biopsy CT scan cervical, thorax, abdominal

 Lymph node biopsy diffuse malignant lymphoid infiltrate, with large medium cells. IHC on lymph node : CD20 positive on tumor cells, bcl2 positive in tumor cells, CD10 negative, Ki67 positive > 90%, CD 30 negative in tumor cells

 BM biopsy hypercellular; important hyperplasia of erythroblastic lineage normoblastic.

IHC on bone marrow - no malignant lymphoid infiltrate

 CT scan : - cervical multiple adenopathies - thorax no adenopathies, no lesions of lungs - abdominal normal liver and spleen, no adenopathies; small gall stone. - pelvic no adenopathies.

What is the diagnosis ? 1. Hodgkins disease 2. Diffuse large B-cell lymphoma 3. Follicular lymphoma 4. Acute lymphoblastic leukemia

 Diffuse large B-cell lymphoma

 Do u need to stage the disease ? Why is the staging important ?

Complete diagnosis

Diffuse large B-cell NH lymphoma stage II A

Therapeutic options
Supportive therapy : transfusions with special red cell mass, corticotherapy, folic acid Specific therapy : chemotherapy + monoclonal antibodies anti CD 20 ( Rituximab)

Evaluation after 3 RCHOP cycles

Clinical examination no adenopathies Cell count : Hb=13 g/dl, Ht=39%, WBC=5300/mmc, PLT= 269.000/mm. Reticulocytes 1% ESR=20 mm per hour TB 1,2 mg/dl, UB 0,7 mg/dl, CB 0,5 mg/dl, LDH 200 U/l.

Evaluation after completing 6 cycles

Clinical good status Normal cell counts, normal biochemistry Normal CT scan

Long term management

Maintenance therapy with Rituximab every 2 month Monitoring cell counts every month When hemolysis transfusion, corticotherapy Folic acid continuously Good hydration, allopurinol No vaccine Treatment of complications of anemia and NHL

Prognosis
Good because of stage II A; good therapy response
Worse because of the onset with AIHA ; possible complications in the future INTERMEDIATE

Particularity of the case

Onset with AIHA

 GOOD LUCK !