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181

A 26-year-old previously well woman on no medication presents with the recent-onset of easy bruising.  She has two small oral mucosal haemorrhages, scattered bruises and petechiae around the ankles but no other abnormalities on examination. 
 
Human immunodeficiency virus (HIV) antibody test is negative. 
 
The full blood count shows: 
  

Prothrombin time-international normalised ratio (PT-INR) and activated partial thromboplastin time (APTT) are normal and bone marrow examination is also normal except for an increase in megakaryocytes. 
 
The most appropriate initial therapy is: 
 
A. intravenous gamma globulin. 
 
B. oral tranexamic acid. 
 
C. platelet transfusion. 
 
D. oral prednis(ol)one. 
 
E. oral danazol. 

D

182

A 23-year-old woman presents with several days history of petechiae of the skin and oral mucosa.  There is no history of infection or medication apart from the oral contraceptive pill.  The patient is well and examination is unremarkable except for the petechiae.   
 
Investigations reveal: 

  

Which one of the following is the most appropriate treatment? 
 
A. Oral prednis(ol)one. 
 
B. Intravenous immunoglobulin. 
 
C. Platelet transfusion. 
 
D. Danazol. 
 
E. Splenectomy. 

A

183

What is the cause of red cell aplasia?

What are the characteristics of the anaemia?

What is the treatment?

Idiopathic and autoimmune (mediated by T-lymphocytes or IgG antibody to erythroid precursors.  
Viral: (parvo, herpes, HIV and hepatitis)
Thymoma
Other malignancy

Drugs:  EPO (alpha aka Eprex), MMF

Severe and normochromic with absent retics

BMBx:  All non- erythroid elements are present and normal, but erythroid precursors are markedly reduced or absent. In some cases, chest imaging studies will reveal a thymoma.

Combination of antithymocyte globulin and a calcineurin inhibitor (cyclosporine or tacrolimus)—similar to therapy of aplastic anemia.

184

What is the mechanism of EPO related red-cell aplasia?

Neutralising antibodies, and will cross-react with all currently available erythropoietic agents (epoetin alfa and beta, and darbepoetin alfa), in addition to endogenous EPO.

185

In which conditions are spherocytes found?

Spherocytes are found in hereditary spherocytosis and autoimmune hemolytic anemia.

186

What is a ringed sideroblast?

Ring sideroblasts are named so because of the arrangement of the iron granules in a ring form in mitochondria around the nucleus of an erythroid precursor

The body has iron available but cannot incorporate it into hemoglobin, which red blood cells need to transport oxygen efficiently.

187

What are the diagnostic essentials of myelodysplastic syndrome?

- cytopaenia with hyper cellular BM (but not more than 20% blasts otherwise it's AML)
- morphologic abnormalities in two or more cell lines

188

What are the causes of myelodysplastic syndrome?

Idiopathic / cytotoxic CTx, RTx or both
Benzene

Red cell aplasia and Fanconi's anaemia can transform to MDS

189

What is the classification of stages of MDS?

Refractory anaemia - no excess bone marrow blasts, may or may not have ringed sideroblasts

Refractory anaemia w/ excess blasts

Chronic myelomonocytic leukaemia - MDS + peripheral blood monocytosis

Prognosis worsens with each.

190

What is 5q- syndrome?

Myelodysplasia with:
5q- syndrome (indolent disease)
Refractory anaemia with loss of long arm of chromosome 5
Good prognosis

191

How is the severity and survival of myelodysplasia ranked?

IPSS - survival and rate of progression AML
Survival related to:
Percentage of bone marrow blasts 0-10% mild, 21-30% is poor
Karyotype:  Normal, Y-, 5q-, 20q- = good
Bad:  Cr 7 (monosomy 7), or if there are 3 or more abnormalities
Cytopaenias - None or 1 cell line is good, 2 or 3 is bad.

192

What are the clinical findings in MDS?

Clinical findings
Age >60
Incidental finding or fatigue/infection/bleeding due to bone marrow failure
Fever, weight loss, general debility
Splenomegaly, pallor and bleeding

193

What are the lab findings in myelodysplasia?

Marked anaemia w/ normal or elevated MCV
Low WCC and neutropenia
Smear:  Macro-ovalocytes, bilobed neutrophils (Pelger Huet abnormality), myeloid left shift with small numbers promyelocaytes or blasts, normal or reduced hypo granular platelets
BMBx:  Erythroid hyperplasia, megaloblastosis:  nuclear budding, multinucleate erythroid precursors
Left shifted myeloid series, characteristic:  dwarf megakaryocytic with a unloved nucleus
Prussian blue stain:  ringed sideroblasts

194

How do you differentiate myelodysplasia from MDS?

AML:  MDS has <20% blasts, AML has >20%

195

What is the treatment of myelodysplasia?

EPO if anaemic if EPO levels are low
G-CSF may help response to EPO.
Highest transfusion requirements = least likely to respond
Lenalidomide for transfusion dependent anaemia due to MDS esp for 5q- syndrome (less success w/o it).  SEs:  neutropenia, thrombocytopaenia, VTE - must use aspirin prophylaxis.  Not useful for other cell lines.
Iron chelation if transfusion dependent.
High risk - azacitidine

196

What is the prognosis of myelodysplasia

Ultimately fatal, cure rates of 30-60%
5q- 5 year survival over 90% or low risk disease
Excess blasts or CMML = higher chance of AML and short survival w/o Allo SCT.

197

What are the diagnostic essentials of hairy cell leukaemia?

Pancytopenia.
Splenomegaly, often massive.
Hairy cells present on blood smear and especially in bone marrow biopsy

198

What is hairy cell leukaemia?

Rare mature b-lymphocytosis with hairy cytoplasmic projections.

199

What are the signs and symptoms of hairy cell leukaemia?

Middle aged men, 55 years 5:1 M:F
Fatigue
Sx related to markedly enlarged spleen
Recurrent infection inc mycobacterial infections
Hepatomegaly in 50%
No lymphadenopathy

200

What are the lab findings in hairy cell leukaemia?

Pancytopaenia (hallmark)
Anaemia (universal), thombocytopaenia and neutropenia
Smear: ‘hairy cells’ B-lymphocytes with cytoplasmic projections
BMBx:  dry tap
Splenic infiltrate of red pulp with hair cells (lymphoma it’s the white pulp)
Immunophenotyping:  CD11c, CD20, Cd22, CD25, CD103, and CD123

201

What is the treatment of hairy cell leukaemia?

IFN-a
Cladribine (purine analogue)
Pentostatin

202

What is the prognosis of hairy cell leukaemia?

Good - >95% of patients are alive longer than 10 years
If secondary to CLL - worse prog

203

What are the diagnostic essentials of Waldenstrom's macroglobulinaemia?

Monoclonal IgM paraprotein
BM infiltration by plasmacytic lymphocytes
Absence of lytic bone disease

204

What is Waldenstrom's macroglobulinaemia?

IgM hypergammaglobulinaemia in setting of NHL.
B-lymphocytes that are a morphological hybrid of lymphocytes and plasma cells.
Cells secrete IgM paraprotein.

205

What are the clinical findings of Waldenstrom's macroglobulinaemia?

Insidious onset in 60s-70s
Fatigue related to anaemia
Hyperviscosity 
--> mucosal and GI bleeding, retinal vein engorgement due to engorged blood vessels and platelet dysfunction
--> neuro sx, nausea, vertigo, visual changes, stupor, coma
--> cold agglutinin disease (haemolysis), chronic demyelinating peripheral neuropathy

Hepatosplenomegaly
Purpura
No bone tenderness
No renal disease
No lytic lesions

206

What are the lab findings in Waldenstrom's macroglobulinaemia?

Anaemia, roleaux, RBC agglutination at rom temp
Anaemia is secondary to expansion of plasma volume (by 50-100%
Plasmacytic lymphocytes on smear
BMBx: Infiltration of plasmacytic lymphocytes
Hallmark:  Monoclonal IgM spike on serum PeP in the B-globulin region
High serum viscosity (symptoms develop at 4x water viscosity, severe at 6x
Positive Coombs for complement
Cold agglutinin or cryoglobulin properties

207

Why and how should you repeat protein electrophoresis if a suspected test for Waldenstrom's macroglobulinaemia comes back with hypogammaglobulinaemia?

Temp must be maintained at body temp of 37c or the paraprotein may precipitate out.  Sample should recollected and kept warm.

208

What is the differential dx of Waldenstrom's macroglobulinaemia?

MGUS --> W has monoclonal malignant cells
CLL --> Absence of CD5, different morphology, absence of lymphocytosis
Multiple myeloma --> BM morphology, IgM paraprotein, absence of bone and renal disease

209

What is the treatment of Waldenstrom's macroglobulinaemia?

Hyperviscosity syndrome (stupor, coma, pulm oedema) --> emergency --> plasmapheresis
Otherwise
Periodic plasmapheresis
FCR (Fludarabine, cyclophosphamide, rituximab)

210

What treatment does all indolant malignant lymphoid diseases have in common?

Rituximab (anti-CD20 antibody)