Severe anemia as an unusual finding in Waldenström’s

Macroglobulinemia: A Case Report

Gerald Abraham1, Alvira Rozalina1, Ikhwan Rinaldi2, Cosphiadi Irawan2

1Department of Internal Medicine, Medical Faculty University of Indonesia-Cipto Mangunkusumo General Hospital
2Division of Hematology-Medical Oncology, Department of Internal Medicine, Medical Faculty University of Indonesia-Cipto Mangunkusumo General
INTRODUCTION RESULT Hospital
.
Lymphoplasmacytoid
Waldenström’s macroglobulinemia (WM) is a rare AIHA Ig M Kappa Monoklonal
lymphocytes
disease that accounts for 1%-2% of non-Hodgkin
lymphomas. WM is a disease of the elderly, with
the median age at the time of diagnosis being 63 –
75 years in different series.1,2 Anemia is one of the
cardinal findings and may be the only indication for
Hepatosplenomegaly
therapy in WM. Severe anemia in WM is an
uncommon finding.3,4

CASE

A 68-year-old man came to the emergency DISCUSSION

department with chief complaint of fatigue. The
patient told that he has been diagnosed with
anemia since 2 years ago but the cause of
anemia has not been found. He was only given
packed red cell transfusion.
Physical examination showed pale conjunctive
and splenomegaly Schuffner 4
Anemia, one of the cardinal findings in Waldenström macroglobulinemia, is present in over 80% of
those patients who are not on “watch and wait.” In some instances, the severity of anemia is mild and
does not require intervention, while in others it is the major reason for designating a patient as being
symptomatic and requiring treatment of Waldenström macroglobulinemia.
Rarely, patients with Waldenström macroglobulinemia can have cold agglutinin hemolytic anemia. This
is a disorder where the IgM protein results in damage to the red blood cell. Damaged red blood cells
are subsequently removed from the circulation in the liver and in the spleen. The mechanism is more
complex than direct Waldenström involvement of the bone marrow, and thus the therapy is both more
complex and often more frustrating.

Indications For Initiation Of Therapy In Patients With WM

Clinical indications for initiation of therapy
Recurrent fever, night sweats, weight loss, fatigue √
Hyperviscosity
Lymphadenopathy: either symptomatic or bulky ( 5 cm in maximum diameter)
The Hemoglobin level was 2.4 gr/dl and blood Symptomatic hepatomegaly and/or splenomegaly √
smear examination showed pancytopenia. Symptomatic organomegaly and/or organ or tissue infiltration √
Peripheral
Table neuropathy due to WM of Procedure
2. The Characteristic
Reticulocyte count showed a hypo proliferative
Laboratory indications for initiation of therapy
marrow. Other laboratory data showed increase Symptomatic cryoglobulinaemia
Symptomatic cold agglutinin anaemia √
lactate dehydrogenase and unconjugated
Autoimmune haemolytic anaemia and/or thrombocytopaenia √
bilirubin. Coombs test examination was positive. Nephropathy-related to WM
Amyloidosis-related to WM
There was inverse albumin globulin ratio, and
Hb 10 g/dL √
serum electrophoresis protein indicate a Platelets < 100109/L √
IgM levels 6000 mg/L √
monoclonal gammopathy. A monoclonal IgM
kappa antibody was confirmed from
immunofixation test. The titer of IgM antibody was CONCLUSION
2495 mg/dL. Patient then underwent bone marrow Not all patients with a diagnosis of WM need immediate therapy. Criteria for the initiation of therapy
biopsy and the result showed hypercellularity of (proposed in the IWWM-2 consensus panel and confirmed in IWWM-8) include IgM related
bone marrow which consists of diffuse small complications and/or symptoms related to direct tumor cells BM involvement such as cytopenias,
lymphocyte cell and plasma cell in between. From constitutional symptoms and bulky extramedullary disease. Some symptoms that need urgent therapy
those findings, we conclude the patient had a such as symptomatic hyperviscosity, moderate to severe hemolytic anemia, and symptomatic
Waldenström’s macroglobulinemia. cryoglobulinemia.

REFERENCE
1. Morie A. Gertz, DIAGNOSIS AND MANAGEMENT OF ANEMIA IN WALDENSTRÖM Macroglobulinemia. IWMF torch, volume 15.3 (august 2014) pages 11, 35.
2. E. Kastritis, et al, Waldenstro¨ m’s macroglobulinaemia: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up, Annals of Oncology (Supplement 0): iv1–
iv10, 2018.doi:10.1093/annonc/mdy146
3. Véronique Leblond, et al, Treatment Recommendations for Waldenström Macroglobulinemia from the Eighth International Workshop on WM. Blood First Edition Paper,
prepublished online July 18, 2016; DOI 10.1182/blood-2016-04-711234
4. Prashant Kapoor , et al, Diagnosis and Management of Waldenström Macroglobulinemia:Mayo Stratification of Macroglobulinemia and Risk-Adapted Therapy (mSMART)
Guidelines 2016, JAMA Oncol. 2017 September 01; 3(9): 1257–1265. doi:10.1001/jamaoncol.2016.5763.
Key Words : Waldenström’s macroglobulinemia, anemia.